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Molecular heterogeneity has confounded attempts to target individual pathways in brain tumors. However, gliomas with BRAF mutations have been identified as being uniquely vulnerable to targeted therapies. Such mutations are predominantly seen in brain tumors of the adolescent and young adult population. Given that accurate and timely identification of such mutations is essential for offering appropriate treatment, treatment centers should offer both immunohistochemical and sequencing methods for detection of these mutations to guide treatment. Additional studies of these tumors at recurrence would also allow identification of breakthrough resistance mechanisms that may also be targetable for treatment. Due to the relative rarity of these tumors, multicenter collaborative studies will be essential in achieving long term control of these tumors.

Posterior reversible encephalopathy syndrome (PRES) is a rare neurologic disorder that presents with variable symptoms and symmetrical abnormal white matter signaling most commonly of the occipital and parietal lobes on magnetic resonance imaging (MRI). PRES, also known as reversible posterior leukoencephalopathy syndrome (RPLS) is commonly associated with hypertension. Hypomagnesemia's association with PRES has been rarely reported. Here, we report a patient with severe hypomagnesemia that presented with PRES syndrome that improved with magnesium replacement. Hypomagnesemia should be considered an underlying etiology in patients presenting with PRES syndrome and should be promptly treated. The presentation can often be concerning for acute cerebrovascular accidents with symptoms of dysarthria and upper motor neuron symptoms, such as facial droop, dysarthria, and gait instability. Differential diagnosis of PRES often includes rostral brainstem infarction, transient ischemic attack, infectious encephalopathy, and metabolic/toxic encephalopathy, which is evaluated in the description of the case. The most common presentation of RPLS/PRES includes altered mental status, drowsiness, seizure, vomiting, alterations in speech including dysarthria, and visual disturbance. The first signs noted are commonly lethargy and somnolence. In this case, the patient presented notably with initial symptoms of dysarthria of speech and facial droop, with serum hypomagnesemia in which symptoms corrected rapidly with the administration of intravenous magnesium sulfate.

Syphilis incidence has increased in the US in the last decade. Jarisch-Herxheimer reaction (JHR) is a well-documented adverse effect of penicillin treatment in syphilis. Stroke has not been reported as part of its phenomenology. Case Report. A 57-year-old man presented with worsening memory. His minimental status examination score was 14/30. Serum RPR test was positive and VDRL test in the CSF was reactive. Within six hours of first dose of IV crystalline penicillin G, he was found to have hemineglect and difficulty moving the left leg. MRI of the brain showed multiple acute ischemic strokes. Immediate MRA ruled out vascular occlusion. Penicillin treatment was stopped. Four hours later, he was found to be febrile and had two episodes of generalized tonic-clonic seizures. Conclusions. We report a case of confirmed neurosyphilis with no known modifiable stroke risk factors, who developed acute ischemic stroke and other constitutional symptoms consistent with JHR after IV penicillin. This is the first reported case in literature where an acute ischemic stroke can be attributed to Jarisch-Herxheimer reaction. Given an increase in incidence of syphilis in recent years, our case underlies the importance of keeping in mind potential catastrophic drug adverse reactions in neurosyphilis patients.

Background There is practice heterogeneity in the use, type, and duration of prophylactic antiseizure medications (ASMs) in patients with moderate–severe traumatic brain injury (TBI). Methods We conducted a systematic review and meta-analysis of articles assessing ASM prophylaxis in adults with moderate–severe TBI (acute radiographic findings and requiring hospitalization). The population, intervention, comparator, and outcome (PICO) questions were as follows: (1) Should ASM versus no ASM be used in patients with moderate–severe TBI and no history of clinical or electrographic seizures? (2) If an ASM is used, should levetiracetam (LEV) or phenytoin/fosphenytoin (PHT/fPHT) be preferentially used? (3) If an ASM is used, should a long versus short (> 7 vs. ≤ 7 days) duration of prophylaxis be used? The main outcomes were early seizure, late seizure, adverse events, mortality, and functional outcomes. We used Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology to generate recommendations. Results The initial literature search yielded 1998 articles, of which 33 formed the basis of the recommendations: PICO 1: We did not detect any significant positive or negative effect of ASM compared to no ASM on the outcomes of early seizure, late seizure, adverse events, or mortality. PICO 2: We did not detect any significant positive or negative effect of PHT/fPHT compared to LEV for early seizures or mortality, though point estimates suggest fewer late seizures and fewer adverse events with LEV. PICO 3: There were no significant differences in early or late seizures with longer versus shorter ASM use, though cognitive outcomes and adverse events appear worse with protracted use. Conclusions Based on GRADE criteria, we suggest that ASM or no ASM may be used in patients hospitalized with moderate–severe TBI (weak recommendation, low quality of evidence). If used, we suggest LEV over PHT/fPHT (weak recommendation, very low quality of evidence) for a short duration (≤ 7 days, weak recommendation, low quality of evidence).

Cerebral air embolism (CAE) is a potentially fatal iatrogenic complication related to common procedures including central venous catheter (CVC) removal. We report an interesting case of CAE related to CVC removal that was further complicated with status epilepticus. Neuroimaging of CAE and status epilepticus could pose diagnostic dilemmas and require consideration of wide diagnostic differentials. We discuss the clinical presentation, mechanism, and diagnostic approach, especially neuroimaging to differentiate various etiologies in CAE patients.

To understand the current state of neurology residents training in neuropathology, we electronically distributed a 16-item survey to 150 adult and 70 child neurology program directors (PDs). The survey inquired about their program characteristics, neuropathology curriculum and assessment methods, trainee performance, and attitudes. Descriptive analysis was used to summarize categorical variables as frequencies and percentages and continuous as means and standard deviations. We conducted a series of Mann-Whitney U and Fisher’s exact tests to evaluate differences between various program characteristics. Sixty-four (29%) PDs responded to the survey, including 45 (30%) adult and 19 (27%) child neurology PDs. Thirty-one programs required a dedicated neuropathology rotation. The majority (92%) used the Residency In-Service Training Examination (RITE) to assess trainee’s knowledge. Approximately 86% of the PDs agreed that neuropathology is essential and a defined curriculum is necessary during residency training. There was no difference in the RITE scores between programs. We conclude that a neuropathology rotation was felt to be essential even though the RITE scores did not differ between programs with and without a dedicated rotation. Alternative evaluation and training methods may need consideration. A future survey of all the stakeholders may be required to thoroughly understand and disseminate the neuropathology education well.

Background There is practice heterogeneity in the use, type, and duration of prophylactic antiseizure medications (ASM) in patients hospitalized with acute nontraumatic intracerebral hemorrhage (ICH). Methods We conducted a systematic review and meta-analysis assessing ASM primary prophylaxis in adults hospitalized with acute nontraumatic ICH. The following population, intervention, comparison, and outcome (PICO) questions were assessed: (1) Should ASM versus no ASM be used in patients with acute ICH with no history of clinical or electrographic seizures? (2) If an ASM is used, should levetiracetam (LEV) or phenytoin/fosphenytoin (PHT/fPHT) be preferentially used? and (3) If an ASM is used, should a long (> 7 days) versus short (≤ 7 days) duration of prophylaxis be used? The main outcomes assessed were early seizure (≤ 14 days), late seizures (> 14 days), adverse events, mortality, and functional and cognitive outcomes. We used Grading of Recommendations Assessment, Development, and Evaluation methodology to generate recommendations. Results The initial literature search yielded 1,988 articles, and 15 formed the basis of the recommendations. PICO 1: although there was no significant impact of ASM on the outcomes of early or late seizure or mortality, meta-analyses demonstrated increased adverse events and higher relative risk of poor functional outcomes at 90 days with prophylactic ASM use. PICO 2: we did not detect any significant positive or negative effect of PHT/fPHT compared to LEV for early seizures or adverse events, although point estimates tended to favor LEV. PICO 3: based on one decision analysis, quality-adjusted life-years were increased with a shorter duration of ASM prophylaxis . Conclusions We suggest avoidance of prophylactic ASM in hospitalized adult patients with acute nontraumatic ICH (weak recommendation, very low quality of evidence). If used, we suggest LEV over PHT/fPHT (weak recommendation, very low quality of evidence) for a short duration (≤ 7 days; weak recommendation, very low quality of evidence).