Multiple Cranial Neuropathies as the Initial Presentation of Primary Ductal Adenocarcinoma of the Lacrimal Gland.

Primary ductal adenocarcinoma of the lacrimal gland is a rare, aggressive malignancy that clinically and histologically resembles salivary duct carcinoma. Similar to other malignant epithelial lacrimal gland tumors, ductal adenocarcinoma typically presents with unilateral proptosis, pain, upper eyelid swelling, palpable mass, diplopia, ptosis, and blurred or decreased vision. Rarely, primary malignant epithelial lacrimal gland tumors may first present with multiple cranial neuropathies due to occult spread to the cavernous sinus, as in this case. With such a vast differential diagnosis, a practical yet systematic approach to multiple cranial neuropathies, as guided by clinical history, exam, and neuroimaging, allows for a more targeted diagnostic evaluation, especially when multiple diagnostic tests and interventions return unrevealing. A repeat biopsy or complete excision of the lacrimal gland may be necessary to yield the correct diagnosis.

Loss of Vestibular Ocular Reflex in Nonconvulsive Status Epilepticus.

BACKGROUND: Electroencephalogram (EEG) findings of generalized periodic discharges (GPDs) with triphasic morphology were introduced as a metabolic phenomenon, but more recently have been associated with epileptic phenomenon. Resolution of EEG findings along with clinical improvement from treatment is diagnostic. The known causes of reversible, isolated loss of OVR include medication toxicity, lead exposure, and thiamine deficiency, but its association with nonconvulsive status epilepticus (NCSE) has never been published. Medication induced loss of OVR resolves after a 24-hour washout period. We report a case of reversible, isolated loss of vestibular ocular reflex (VOR) associated with epileptic phenomenon. METHODS: This is a case report of a single patient. RESULTS: A 74-year-old male with a history of complex partial seizures admitted for a pneumonectomy had a post-operative course complicated by two instances of coma, the latter associated with an isolated loss of VOR. EEG revealed GPDs with triphasic morphology initially interpreted as a metabolic phenomenon. The patient's mental status, exam and EEG findings improved after low dose infusion of propofol for tracheostomy, and he was eventually discharged at baseline neurological function. Due to this response, his coma, loss of VOR and EEG were later interpreted as a consequence of NCSE. CONCLUSION: The interpretation of GPDs with triphasic wave morphology range from metabolic phenomenon to NCSE. NCSE should be highly considered on the differential for encephalopathy regardless of the circumstances. NCSE may be a potential cause of reversible, isolated loss of the VOR and an AED trial in the appropriate clinical context should be considered. This is the first report of loss of VOR possibly associated with NCSE.

Early cerebral venous drainage associated with focal seizures: a correlation of digital subtraction angiography and electroencephalography. Illustrative case.

BACKGROUND: Early venous drainage is a critical diagnostic feature of arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs). However, other conditions associated with early venous drainage can mimic AVMs and AVFs and mislead the treating physician team. OBSERVATIONS: The authors present a unique case of a 56-year-old man with new left hemianopsia. Workup with magnetic resonance imaging and magnetic resonance angiography had revealed cortical enhancement and early venous drainage, possibly caused by an AVF or AVM. Catheter angiography confirmed the presence of early venous drainage of specifically the right occipital lobe. The early venous drainage occurred as the result of local hyperperfusion limited to the right occipital lobe. Subsequent electroencephalography confirmed focal seizure activity in the right occipital lobe. After seizure control with multiple antiepileptic medications, the patient regained his vision. Repeat digital subtraction angiography revealed restoration of normal cerebral blood flow. LESSONS: Early venous drainage observed during catheter angiography can be associated with seizure activity. Differentiation from other conditions, such as AVM, AVF, or ischemic stroke, is critical to facilitate prompt and appropriate treatment.

Neuropsychological normative standards for late career physicians.

Objective: In the context of an aging, vital physician workforce, there is clear value in establishing a specialized neuropsychological normative dataset for the evaluation of late career physicians practicing clinical medicine. Methods: Physicians aged 60 and over in active clinical practice at 3 major medical centers in North Carolina were recruited to complete a comprehensive neuropsychological test battery. Results: The sample (n = 100) was aged 60-78. Reflecting medical school distributions for this age group, the sample was mostly male (76%) and was fairly racially homogenous (96% White). To amplify utility of the dataset, data were obtained for several measures across neurocognitive domains. Results are presented in percentile bands stratified by age and sex where needed, and regression formulas are presented for predictive precision for measures where both age and sex predicted performance. Important distinctions between our sample, the general population, and current comparative demographic norms were also confirmed. Conclusions: Here we present a preliminary normative dataset on a comprehensive neuropsychological test battery for late career physicians, the HEATS norms for highly educated aging treaters. These standards facilitate accurate, objective cognitive measurement as a part of clinical diagnostic and capacity evaluations. Co-norming the battery in a single, specialized sample further augments its utility in characterizing cognition. Future work to enhance the dataset to be maximally applicable across demographic groups is anticipated, as is research to explore the relationship between these standards and professional performance outcomes.

Yoga and Mindfulness Intervention for Concussion Recovery.

Concussion imposes a substantial global health burden, and few evidence-based treatments that approach concussion treatment holistically are available. Moreover, early intervention is important before concussion symptoms become chronic and more refractory to treatment. In this pilot study, we evaluated the tolerability of a protocol called Mindfulness and Yoga for Treatment After Concussion (MYTAC), which was specifically designed for individuals with concussion. We also explored the possible benefit of the MYTAC protocol for concussion recovery. Participants were 15-60 years old with recent concussion and had presented to selected practices within a university health system. Participants used the video-based MYTAC protocol for 5 consecutive days and reported concussion symptoms on an abbreviated version of the Sports Concussion Assessment Tool version 3 (SCAT3). We compared the abbreviated SCAT3 scores across the intervention period, including immediately before and after each yoga session, using standard statistical methods for paired data. Of 25 participants enrolled in the study, 15 completed the MYTAC protocol and 1 completed 2 days of the protocol before withdrawing due to worsening symptoms; the remaining 9 participants did not complete the protocol. On average, total abbreviated SCAT3 scores decreased by 9.9 ± 7.6 points, or approximately 50%, over the intervention period (from an initial value of 18.8 ± 6.7 points prior to the yoga protocol). Although this pilot study had significant methodological limitations, we concluded that the MYTAC protocol had fair tolerability and possibly a beneficial effect on concussion recovery. However, future interventions should evaluate this protocol in larger, more rigorously designed studies.

Something Got Your Tongue? A Unique Cause of Hypoglossal Nerve Palsy.

Introduction: The authors report a rare cause of isolated hypoglossal nerve palsy caused by a high cervical osteophyte. This case increases clinical knowledge of an underreported condition and teaches the clinician radiologic pearls in making the diagnosis. To their knowledge, this is the first case report showing surgical remediation of this condition. Symptoms and Clinical Findings. A 73-year-old female presented with several months of occipital headache, progressive dysarthria, dysphagia, and tongue deviation to the right. Her neurologic exam was significant for atrophy of the right hemitongue with tongue fasciculations. On protrusion, her tongue deviated rightward. Diagnosis and Therapeutic Intervention. Careful review of her initial head computed tomography (CT) imaging revealed that a high cervical osteophyte caused unilateral, isolated hypoglossal nerve palsy. Neurosurgery performed a right, far lateral approach for decompression of this osteophyte and over the ensuing months her symptoms improved. Conclusion: High cervical osteophyte is an underrecognized cause of isolated hypoglossal nerve palsy. The imaging investigation should be systematic and focus on the skull base with magnetic resonance imaging (MRI) or CT. This is a rare occasion when high resolution CT of the skull base can actually be the more helpful imaging modality. As shown in this case, an osteoarthritic cause can be surgically ameliorated.

Time to diagnosis in the National Registry of Veterans with Amyotrophic Lateral Sclerosis.

Our objective was to determine the interval from symptom onset to diagnosis, and to evaluate associated factors in a cohort of U.S. Veterans with motor neuron diseases. We retrospectively evaluated 1359 patients enrolled in the National Registry of Veterans with Amyotrophic Lateral Sclerosis (ALS). The main outcome measures were time from symptom onset to first diagnosis and to second opinion. Predictor variables included age at symptom onset, year of symptom onset, race, onset site, final diagnosis, number of diagnostic tests performed and clinical sites visited. Median time to first diagnosis was 11 months; median time to second opinion was two months. In a multivariable model, more recent calendar year of symptom onset, younger age, bulbar onset and a diagnosis of ALS versus non-ALS motor neuron disease were all significantly associated with a shorter time to first diagnosis. Later year of symptom onset and white race were significantly associated with a shorter time to second opinion. While the interval from symptom onset to diagnosis, and many of the associated factors are similar between our large cohort of U.S. Veterans with ALS and other smaller published cohorts, we found that the diagnostic interval among U.S. Veterans has significantly decreased over time.

Adult cases of leukoencephalopathy, cerebral calcifications, and cysts: expanding the spectrum of the disorder.

Leukoencephalopathy with cerebral calcifications and cysts (LCC) was first reported in children who developed cognitive decline and variable extrapyramidal, cerebellar, and pyramidal signs, with or without seizures. Leukoencephalopathy with cerebral calcifications and cysts is characterized by progressive formation of brain cysts that can generate a mass effect simulating a neoplasm. Retinal changes that overlap with Coats disease, a microangiopathy with retinal telangiectasias and exudates, may also occur. We and others have reported LCC cases in adults. Neuroimaging shows diffuse leukoencephalopathy, multifocal calcifications especially of deep gray and white matter, multifocal enhancement, and variably sized cysts that may require surgical decompression. Biopsies adjacent to cysts have shown angiomatous and/or severely hyalinized blood vessels surrounded by myelin loss and gliosis, calcifications, and Rosenthal fibers. We report 2 additional adult-onset cases of LCC. Case 1 is a 40-year-old man who developed neurological symptoms and cirrhosis and died of acute gastrointestinal bleeding; he had numerous retinal microinfarcts at autopsy. Case 2 is a 55-year-old woman who was found by chance to have LCC; one and a half years later, her course remains benign. These cases expand the spectrum of adult-onset LCC, the etiology of which is unknown.

Factors associated with survival in the National Registry of Veterans with ALS.

The clinical course of patients with ALS is highly variable. While the median survival time from symptom onset is 2-4 years, there are reports of survival ranging from less than a year to more than 40 years. Such variability makes planning difficult for patients and physicians, and complicates clinical trial design. We sought to validate previous predictors of survival and search for new ones using a large group of ALS patients in the National Registry of Veterans with ALS. We were especially interested in how various aspects of military service might affect survival. Subjects were those in the National Registry of Veterans with ALS who had probable or definite ALS (according to El Escorial criteria). A multivariable Cox proportional hazard regression model was used to examine variables for statistical association with ventilator-free survival time (determined from date of first diagnosis). Subjects who had not died or started ventilation by 31 October 2006 were censored. Our group of 1085 US military veterans with ALS was primarily male (98%) and white (94%), with mostly sporadic (95%) and extremity-onset (76%) ALS. Symptom onset occurred at a mean age of 59.3 years (60.6 years for diagnosis). Median survival time from symptom onset was 4.7 years (3.3 years from diagnosis). In our multivariable model, older age at diagnosis (HR 1.41 (95% CI 1.27-1.55) per 10-year increase), non-extremity site of onset (HR 1.55 (1.24-1.94)), and past deployment to Vietnam (HR 1.73 (1.36-2.19)) were all associated with shortened survival. A longer time to diagnosis was associated with better survival (HR 0.77 (0.70-0.84) per one year increase in diagnosis time). In this unique cohort of veterans with ALS, traditional factors of reduced survival remained important. In addition, past deployment to Vietnam was found to be associated with shortened survival as well. This finding could be due to a common exposure, a shared characteristic, an unmeasured confounder, or an enrollment bias. More research will be needed to understand the reasons behind this new finding.

The clinical presentation and treatment of MOG antibody disease at a single academic center: A case series.

OBJECTIVES: To describe the clinical presentation of MOG antibody disease (MOG-AD) in a series of patients at a single academic center. METHODS: We performed a retrospective review of patients with MOG antibodies. RESULTS: We review the clinical presentation of 11 patients with MOG antibodies. In patients seen at Duke University Health System with MOG antibodies, the most common presentation was optic neuritis. Rituximab was the most used treatment for long-term management. CONCLUSIONS: Our case series highlights the common presentation of MOG antibody disease (MOG-AD) at a single academic medical center.

Emerging Subspecialties in Neurology: Sports neurology training and certification: An overview in 2018.

Sports neurology is a relatively new specialty in neurology. Interested trainees may find it difficult to learn about issues in sports neurology both in medical school and residency. This review highlights what the current state is in medical school and residency training and how an interested trainee or neurologist could pursue a career in this area.

Nivolumab-Induced Autoimmune Encephalitis in Two Patients with Lung Adenocarcinoma.

Immune checkpoint inhibitors have improved patient survival outcomes in a variety of advanced malignancies. However, they can cause a number of immune-related adverse effects (irAEs) through lymphocyte dysregulation. Central nervous system (CNS) irAEs are rare, but as the number of indications for checkpoint inhibitors increases, there has been emergence of CNS immune-mediated disease among cancer patients. Given the relatively recent recognition of checkpoint inhibitor CNS irAEs, there is no standard treatment, and prognosis is variable. Therefore, there is a great need for further study of checkpoint inhibitor-induced CNS irAEs. Here, we present two unique cases of nivolumab-induced autoimmune encephalitis in patients with non-small cell lung cancer and review the available literature.

Friday night palsy: an unusual case of brachial plexus neuropathy.

Brachial plexopathy can result from traction injury, radiation injury, local or metastatic cancer, hereditary, or idiopathic causes. However, brachial plexopathy resulting from malposition of an arm during sleep, similar to Saturday night palsy, has not been reported. We report a case of brachial plexus neuropathy that occurred after the patient slept on his shoulder and arm following excessive alcohol consumption on a Friday night.

The Duke neurology advanced practice provider residency: Its time has come.

The acknowledgment that specialization in neurology based on a clinician's extensive background in neuroscience contributes to expertise in patient care was a motivating factor in developing neurology residency programs. The increasing demand for more and better access to neurologic care has created a health care environment ripe for innovation. Advanced practice providers currently do not have the opportunity during primary training to gain much experience in neurologic care. We discuss the challenges and benefits of developing a 1-year neurology residency program for nurse practitioner and physician assistant graduates at our institution. We propose that providing advanced practice providers with specialty skills through neurology residency programs such as ours will be integral to meet the growing clinical need for neurologic care.