Sandipan Pati

A CASE OF NEUROSARCOIDOSIS SUCCESSFULLY TREATED WITH RITUXIMAB

Involvement of the CNS is estimated in about 5% of patients with systemic sarcoidosis and less than half manifest isolated CNS disease.1 The diagnosis of definite neurosarcoidosis requires tissue biopsy demonstrating noncaseating granulomas in the absence of organisms or other causes.2 Corticosteroids are the mainstay of therapy and in one case series, over 70% of patients relapsed or progressed despite treatment with corticosteroids and oral immunosuppressant agents.3 Successful use of infliximab in cases of neurosarcoidosis has been described.4 Here we report a case of a patient who failed to respond to steroids and immune therapies and remained stable after treatment with B-cell-depleting therapy. This being a case report of one patient without blinded observation for treatment, it is a Class IV study. ### Case report. A 50-year-old, right-handed white woman presented with clumsiness affecting the right upper extremity. Within a few days, the deficit progressed to complete loss of strength of the right upper extremity. MRI of the brain revealed multiple lesions in the bilateral hemispheres, some of which enhanced postcontrast (figure, A–C). CSF showed 3 cells, protein 47 mg/dL (normal 15–45), glucose 66 mg/dL (normal 40–70), immunoglobulin G index 0.51 (normal 0.28–0.66), synthesis rate 0.0 mg/day (normal 0–8), negative oligoclonal bands, and myelin basic protein 6.63 ng/mL (normal 0.07–4.1). CT chest, abdomen, and pelvis, bone scans, conventional …

Spectrogram screening of adult EEGs is sensitive and efficient

Objective: Quantitatively evaluate whether screening with compressed spectral arrays (CSAs) is a practical and time-effective protocol for assisting expert review of continuous EEG (cEEG) studies in hospitalized adults. Methods: Three neurophysiologists reviewed the reported findings of the first 30 minutes of 118 cEEGs, then used CSA to guide subsequent review (“CSA-guided review” protocol). Reviewers viewed 120 seconds of raw EEG data surrounding suspicious CSA segments. The same neurophysiologists performed independent page-by-page visual interpretation (“conventional review”) of all cEEGs. Independent conventional review by 2 additional, more experienced neurophysiologists served as a gold standard. We compared review times and detection rates for seizures and other pathologic patterns relative to conventional review. Results: A total of 2,092 hours of cEEG data were reviewed. Average times to review 24 hours of cEEG data were 8 (±4) minutes for CSA-guided review vs 38 (±17) minutes for conventional review (p < 0.005). Studies containing seizures required longer review: 10 (±4) minutes for CSA-guided review vs 44 (±20) minutes for conventional review (p < 0.005). CSA-guided review was sensitive for seizures (87.3%), periodic epileptiform discharges (100%), rhythmic delta activity (97.1%), focal slowing (98.7%), generalized slowing (100%), and epileptiform discharges (88.5%). Conclusions: CSA-guided review reduces cEEG review time by 78% with minimal loss of sensitivity compared with conventional review. Classification of evidence: This study provides Class IV evidence that screening of cEEG with CSAs efficiently and accurately identifies seizures and other EEG abnormalities as compared with standard cEEG visual interpretation.

Diagnosis and Management of Epilepsy Associated With Hypothalamic Hamartoma An Evidence-Based Systematic Review

The main objective was to review the evidence for management of epilepsy associated with hypothalamic hamartomas. We performed a systemic review of the literature through July 2012 that studied patients with hypothalamic hamartomas and related epilepsy. Articles meeting selection criteria were rated according to the American Academy of Neurology classification of evidence scheme. Recommendations were linked to the strength of the evidence and as follows: (a) precocious puberty is associated more with the pedunculated type and epilepsy typified by gelastic seizures with the sessile form of hypothalamic hamartomas (class III); (b) significant behavioral and cognitive deficits are associated with patients with hypothalamic hamartomas (class III); (c) video electroencephalography (EEG) findings are extremely variable particularly across the different ages and do not affect surgical outcome (class III); d) various surgical techniques (transcallosal and endoscopic resection) resulted in 49% to 54% seizure freedom, 15% with a pterional approach as well as about 40% with radiosurgery (class III).

Repeat surgery for hypothalamic hamartoma in refractory epilepsy

OBJECT Hypothalamic hamartomas (HHs) often cause pharmacoresistant epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Surgical intervention offers the patient the best opportunity of seizure resolution, which occurs in approximately 50%–60% of patients, and improvement in both cognitive and behavioral difficulties. For those in whom the initial operation has failed, further medical treatment options remain quite limited, whereas, in some cases, a second surgery may improve seizure outcome. The authors retrospectively reviewed their surgical cases to document the success rate and complications of reoperations in patients with HHs. METHODS Data were obtained from the HH epilepsy surgery database at the Barrow Neurological Institute between 2003 and 2010. Surgical treatment consisted of open and endoscopic procedures, as well as radiosurgery. Demographic details, seizure history, presurgical evaluation, and postoperative follow-up data were evaluated. RESULTS In the last 7 years, 21 (13%) of 157 patients underwent reoperation after an initial epilepsy operation. The initial surgical approach in the 21 patients included: endoscopic (8 patients [38%]), transcallosal (8 patients [38%]), orbitozygomatic (3 patients [14%]), and radiosurgery (2 patients [10%]). Of the 8 patients who initially underwent endoscopic resection, repeat procedures included: radiosurgery in 4 (50%), an orbitozygomatic approach in 2 (25%), repeat endoscopy in 1 (12.5%), and a transcallosal approach in 1 (12.5%). Repeat procedures after an initial transcallosal resection included: endoscopic resection in 2 (25%); radiosurgery in 1 (12%); an orbitozygomatic approach in 2 (25%), and repeat transcallosal surgery in 3 (38%). Predominant seizure types that recurred after the first surgery were gelastic seizures, complex partial seizures, and tonic-clonic seizures. Magnetic resonance imaging in all patients prior to reoperation demonstrated either residual HH and/or connection with the mammillary bodies. Review of patients with more than 6 months of follow-up since the last surgery showed greater than 90% reduction in seizures in 4 patients (19%) and by 50%–90% in 10 patients (48%). Two patients were seizure free, and in 5 patients (24%) there was no change in seizure frequency. Following reoperation, none of the patients had any worsened behavioral issues such as increased rage attacks or disruptive violent behavior. New postoperative complications after reoperation included hemiparesis, thalamic stroke (asymptomatic and symptomatic), hyperphagia, and panhypopituitarism. CONCLUSIONS Reoperation should be considered in selected patients with HH in whom initial epilepsy surgery fails because more than half the patients have significant reductions in seizure.

Lennox-Gastaut Syndrome Symptomatic to Hypothalamic Hamartoma: Evolution and Long-term Outcome Following Surgery

BACKGROUND Lennox-Gastaut syndrome is a catastrophic childhood cryptogenic or symptomatic epilepsy. Hypothalamic hamartomas cause refractory epilepsy often consistent with Lennox-Gastaut syndrome. METHODS Children with Lennox-Gastaut syndrome were defined by a triad of multiple generalized seizure types, slow spike-and-wave on EEG, and mental retardation. RESULTS Twenty-one of 159 hypothalamic hamartoma patients (14%) met the diagnostic criteria of Lennox-Gastaut syndrome. The median age of patients at epilepsy onset was 0.9 years (range, birth to 9 years). Six of the 21 patients (28%) had preceding infantile spasms. All patients underwent different surgical approaches, including endoscopic, transcallosal, orbitozygomatic resections, and radiosurgery treatment. Five of the 21 (24%) were seizure free with an additional 9 (42%) having at least >90% seizure reduction. Only 1 patient was not effectively treated (<50% seizure reduction). Eighty-eight percent of parents reported improvement in behavioral functioning. Shorter duration of epilepsy prior to surgery was a significant predictor of surgical outcome. CONCLUSIONS Patients with Lennox-Gastaut syndrome symptomatic to hypothalamic hamartomas have better postsurgical outcome due to other etiologies compared with cryptogenic and symptomatic Lennox-Gastaut syndrome patients. However, compared with overall hypothalamic hamartomas postsurgical outcomes, this cohort was less favorable. Earlier surgery may lead to better outcomes.

Characteristics of falls in the epilepsy monitoring unit: A retrospective study

Falls are an important adverse event in an epilepsy monitoring unit (EMU). We identified patterns of falls in an EMU and compared them with risk factors for inpatient falls. Twenty-six patients with 26 falls (2.3% of admissions) in the EMU were compared with 50 general neurology inpatients with 56 falls over a 4-year period. In the EMU, the majority (62%) of falls happened during the first 3 days of admission, mostly in the bathroom (74%), in patients with a normal mental status (77%). Most general inpatients fell after the third day (64%), inside their rooms (68%), and had an altered mental status before the fall (68%). All 26 EMU patients were identified as high risk at admission, in spite of which falls were not prevented. We outline these differences between EMU patients and general inpatients and highlight the practice gap in preventing falls in an EMU.

How focal is generalized epilepsy: A distinction with a difference?

The term “generalized epilepsy” was used by the first Commission on Classification and Terminology of the ILAE in 1970 to denote an epilepsymanifesting as “generalized seizures, bilateral seizures, or seizures without any local onset” [1]. Since then, experimental studies have challenged this entrenched opinion [2,3]. The lack of a justifiable therapeutic surgical opportunity in generalized epilepsy has limited the exploration of this condition with intracranial electrophysiology. Progress in understanding the anatomical substrate of generalized epilepsy has, therefore, been substantially slowed and has been based mainly on animal studies and a limited armamentarium of noninvasive investigations [4,5]. Among the latter, simultaneous recordings of fMRI and EEG (EEG/fMRI) appear to be a powerful and promising tool. By defining the electroclinical and hemodynamic correlates of EEG activity, fMRI has shed light on some neurophysiological mechanisms underlying epileptic phenomena. In this issue, Kay and Szaflarski have reviewed the role of EEG/fMRI in understanding genetic generalized epilepsies (GGEs). The authors have highlighted pertinent advances in the analysis of EEG/fMRI data, technical limitations of fMRI, and challenges facing EEG/fMRI studies at ultra-high strengths. They have organized their review around four key questions, paraphrased here, and we will organize our comments around the same four questions:

Long-term outcome after emergency resection of hypothalamic hamartomas for status gelasticus

OBJECT Gelastic seizures are epileptic events characterized by bouts of laughter. They are rare and mostly associated with hypothalamic hamartomas (HHs). Status gelasticus, a rare form of status epilepticus, is defined as a prolonged cluster of gelastic seizures (> 20-30 minutes) without necessarily involving loss of awareness between seizures. Emergency resection of the hamartoma is highly effective in these situations and should be considered as early as possible. The authors retrospectively reviewed their surgical cases to document the success, complications, and long-term follow-up after emergency resection of HHs for status gelasticus. METHODS The authors report on a retrospective case series from a single tertiary care center. Three patients who presented with status gelasticus underwent emergency resection of HHs. Demographic details, seizure history, medical treatment, and postoperative follow-up data were evaluated. Long-term follow-up (minimum 2 years) data were obtained either from the last clinic visit notes or via telephone and e-mail contacts. The institutional review board at St. Josephs Hospital approved this study. RESULTS In the last 7 years, of 157 patients who underwent HH resection, the resection was performed on an emergency basis for status gelasticus in 3 cases. At emergency surgery, these 3 patients ranged in age from 9 months to 3.5 years. All of the patients were boys. Delalande and Fohlen Type II, III, and IV lesions were present in the 3 patients. Surgical approaches for resection of HH included an orbitozygomatic, transcallosal anterior interforniceal approach and endoscopic resection. Status gelasticus was terminated following emergency surgery in all cases, and 1 patient was seizure free. Postsurgical complications included, in 1 case, a small right thalamic infarct with mild transient left hemiparesis, which completely resolved within 2 days. Within 2 years of their original surgery, 2 patients underwent further elective surgeries (endoscopic resection and radiosurgery for persistent symptomatic seizures). Follow-up since their most recent surgery ranged from 8 months to 2 years. Two patients were seizure free and 1 patient had greater than 50% reduction in seizures. CONCLUSIONS Status gelasticus associated with HHs can be successfully terminated by emergency resection of the HH. Long-term follow-up in the present series suggests good seizure freedom results or at least greater than 50% reduction in seizures, although repeat operations were necessary.

An enhanced cerebral recovery index for coma prognostication following cardiac arrest.

Prognostication of coma outcomes following cardiac arrest is both qualitative and poorly understood in current practice. Existing quantitative metrics are powerful, but lack rigorous approaches to classification. This is due, in part, to a lack of available data on the population of interest. In this paper we describe a novel retrospective data set of 167 cardiac arrest patients (spanning three institutions) who received electroencephalography (EEG) monitoring. We utilized a subset of the collected data to generate features that measured the connectivity, complexity and category of EEG activity. A subset of these features was included in a logistic regression model to estimate a dichotomized cerebral performance category score at discharge. We compared the predictive performance of our method against an established EEG-based alternative, the Cerebral Recovery Index (CRI) and show that our approach more reliably classifies patient outcomes, with an average increase in AUC of 0.27.

Accuracy of Limited-Montage Electroencephalography in Monitoring Postanoxic Comatose Patients

Background. Continuous EEG (cEEG) monitoring may help to identify the small percentage of adults with hypoxic-ischemic encephalopathy (HIE) who will regain consciousness if allowed sufficient time. However, the limited yield in this population has led some to question the cost-effectiveness cEEG monitoring in this population. We hypothesized that limited-montage cEEG could provide essentially the same neurophysiologic information at lower cost. In this proof of concept study, we aim to demonstrate the potentials of limited channel EEG in prognostication in postanoxic patients. Methods. We retrospectively reviewed cEEG data from cases monitored at our institution with conventional 21-channel EEG over a 6-month period. Twenty-eight cases were identified in which patients with HIE underwent cEEG for at least 24 hours. Gold-standard findings were determined by conventional visual analysis of the full cEEG, and 2 independent electroencephalographers scored the same data using only limited-montage (4-channel) views. The sensitivity and specificity of limited-montage cEEG review were compared with conventional analysis. We also compared the relative costs of conventional and limited-montage EEG. Results. Using 4-channel limited montage cEEG, reviewers were able to classify accurately background continuity (in 88%), background amplitude (in 81%), maximum background frequency (in 70%), periodic epileptiform discharges, including a seizure (in 92%) and sporadic discharges (in 91%). All epileptiform features were detected with greater than 90% sensitivity and specificity. Eye movement artifact seen over bifrontal electrodes gave false positive detections of periodic epileptiform discharges in 31% of cases. Conclusions. Limited-channel continuous EEG monitoring can provide meaningful electrophysiological data that can be used for prognostication in postanoxic comatose patients. Limited channel EEG can be a cost-effective alternative to conventional EEG monitoring in post-anoxic comatose patients.