Sener, Uğur

Abstract OBJECTIVE: We hypothesize that patients with neurocutaneous melanocytosis-associated melanoma and ventriculoperitoneal shunts are at risk of developing intraperitoneal spread of melanoma. BACKGROUND: Neurocutaneous melanocytosis, a rare condition characterized by excessive proliferation and deposition of melanocytes in the leptomeninges and brain parenchyma, typically occurs in children with large congenital melanocytic nevi and multiple smaller congenital nevi. These patients are at heightened risk for developing NRAS+ melanomas in the central nervous system, which in turn may lead to symptomatic hydrocephalus requiring cerebrospinal fluid diversion for symptom relief. METHODS: Retrospective single-institution study of patients with histologically or radiographically confirmed NCM evaluated at Memorial Sloan Kettering Cancer Center (MSKCC) from 2001-2022. RESULTS: Of the 47 patients with a diagnosis of NCM, 44 patients had symptomatic neurological complications. Eleven patients developed hydrocephalus, 10 had CNS melanoma, and required ventriculoperitoneal shunt placement. Nine of the 10 patients ultimately died of their disease. Three patients were diagnosed with intraperitoneal melanoma, though data are unavailable for the remaining eight. CONCLUSIONS: All (n=11) patients with NCM-associated CNS melanoma required VP shunts for symptomatic relief. Ten of these patients died within 4.3 years of VP shunt placement, with a range of 1 month to 13.5 years prior to succumbing to their disease. While the intraperitoneal pathology remains unknown for 7 of the cases, 3 had confirmed intraperitoneal melanoma, suggesting that VP shunts provided the conduit to CNS melanoma seeding of the peritoneum. Obtaining baseline abdominal imaging studies prior to VP shunt placement may be helpful in the follow-up of these patients.

Objective: To perform a feasibility pilot study comparing the usefulness of EEG electrode cap versus standard scalp EEG for acquiring emergent EEGs in emergency department, inpatient, and intensive care unit patients. Background: Nonconvulsive status epilepticus (NCSE) is a neurological emergency diagnosed exclusively by EEG. Nonconvulsive status epilepticus becomes more resistant to treatment 1 hour after continued seizure activity. EEG technologists are alerted “stat” when there is immediate need for an EEG during oncall hours, yet delays are inevitable. Alternatively, EEG caps can be quickly placed by in-house residents at bedside for assessment. Design/Methods: EEG caps were compared with standard-of-care “stat” EEGs for 20 patients with suspected NCSE. After the order for a stat EEG was placed, neurology residents were simultaneously alerted and placed an EEG cap prior to the arrival of the on-call out-of-hospital technologist. Both EEG cap recordings and standard EEG recordings were visually reviewed at 10 and 20 minutes in a blinded manner by two electroencephalographers. The timing, accuracy of interpretation, and diagnosis between the two techniques were then compared. Results: Of the 20 adult patients, 70% (14 of 20) of EEG cap recordings were interpretable, whereas 95% (19 of 20) standard EEGs were interpretable; three had findings consistent with NCSE on both the EEG cap and standard EEG recordings. In the time analysis, 16 patients were included. EEG cap placement was significantly more time efficient than an EEG performed by technologist using the usual “stat” EEG protocol, with the median EEG cap electrode placement occurring 86 minutes faster than standard EEG (22.5 minutes vs. 104.5 minutes; P < 0.0001; n = 16). Conclusions: New rapid EEG recording using improved EEG caps may allow for rapid diagnosis and clinical decision making in suspected NCSE.

Summary: Smartphones and other personal electronic devices present novel cortical processing tasks with potential for identification of novel EEG waveforms. A 17-year-old patient with epilepsy manifested as recurrent myoclonic seizures, absence seizures, and a single generalized tonic–clonic seizure was hospitalized to undergo video-EEG monitoring for seizure quantification and classification of the epilepsy syndrome. During the monitoring session, a frontocentral predominant 5 to 6 Hz theta rhythm was identified only when the patient was actively texting or playing a video game on his smartphone. Previously, patients with focal epilepsy have been found to have a frontocentral theta rhythm on EEG while texting on mobile devices. We report similar EEG findings in a patient with genetic generalized epilepsy during smartphone gaming to expand the population and triggers for this theta waveform. Given the young age and type of epilepsy, we suggest that the waveform represents the EEG manifestation of the attention–visuomotor pathway that is stimulus independent.

ABSTRACT Introduction Needle electromyography (EMG) findings help confirm myopathy and may indicate specific pathologic changes on muscle biopsy. Methods We conducted a retrospective chart review of 218 consecutive patients referred for muscle biopsy. Presence of specific needle EMG findings was correlated with pathologic findings of inflammation, necrosis, splitting, and vacuolar changes. Sensitivity, specificity, and positive and negative predictive values of specific EMG findings for pathologic changes were calculated. Results Short‐duration motor unit potentials (MUP) were sensitive (83%–94%) but not specific (34%–49%) for pathologic changes. Fibrillation potentials were 65%–74% sensitive and 58%–81% specific for inflammation, necrosis, splitting, or vacuolar changes. The absence of fibrillation potentials had high negative predictive value (82%–93%) for inflammation, splitting, or vacuolar changes. Discussion Fibrillation potentials and short‐duration MUPs predict pathologic changes of muscle fiber necrosis, splitting, and/or vacuolar changes (as seen with inflammatory myopathies and muscular dystrophies). Absence of fibrillation potentials suggests other myopathologic changes (e.g., congenital myopathy). Muscle Nerve 59 :315–320, 2019