Neurosyphilis: mighty imitator forays with benign presentation and unique neuroimaging findings.

Background Common causes of temporal lobe hyper intensities are central nervous system infections like herpes simplex encephalitis, Lyme disease, limbic encephalitis and vascular pathology like Cerebral Autosomal Dominant Arteriopathy with Subcortical infarcts and Leukoencephalopathy. METHODS: Personal assessment, laboratory data analysis and neuroimaging for the patient who was admitted to a central Pennsylvania tertiary care referral centre were conducted. RESULTS: A 52-year-old male presented with a 1-year history of diffuse dysesthesia in upper and lower extremities with associated intermittent headaches and neck stiffness. Evaluation with lumbar puncture revealed increased nucleated cells (50ul) with lymphocytic predominance (96%) and an elevated protein level of 109mg/dl. Magnetic resonance imaging (MRI) of the brain showed T2/FLAIR hyper intensity in bilateral subcortical temporal white matter, left-greater-than-right and associated volume loss in cerebral parenchyma. Additional abnormal work up included reactive serum reactive plasma regain and Treponema pallidum antibody particle agglutination. Diagnosis of neurosyphilis was made and the patient was treated with intramuscular (IM) penicillin for 3 weeks. At the time of discharge, his headache and neck stiffness resolved and dysesthesias were decreased in intensity. CONCLUSIONS: The diagnosis of neurosyphilis is intricate, and no reference standard exists. Neuroimaging findings of neurosyphilis commonly are cerebral infarctions, leptomeningeal enhancement or non-specific white matter lesions. Less common features on fluid-attenuated inversion recovery (FLAIR) sequences are cortical atrophy and mesial temporal parenchymal signal changes. It is prudent to keep neurosyphilis in differential of mesial temporal lobe white matter changes, as early diagnosis and treatment results in better prognosis.

Lateralized hyperkinetic motor behavior.

Seizures are followed by a post-ictal period, which is characterized by usual slowing of brain activity. This case report describes a 68-year old woman who presented with right-sided rhythmic, non-voluntary, semi-purposeful motor behavior that started 2 days after an episode of generalized seizure. Her initial electroencephalogram (EEG) showed beta activity with no evidence of epileptiform discharges. Computed tomography scan showed hypodensity in the left parieto-occipital region. Magnetic resonance imaging (MRI) showed restricted diffusion/fluid-attenuated inversion recovery hyperintensities in the left precentral and post-central gyrus. Unilateral compulsive motor behavior during the post-ictal state should be considered, and not confused with partial status epilepticus to avoid unnecessary treatment. Abnormal magnetic resonance imaging (MRI) findings, which are reversible, can help with the diagnostic and therapeutic approach.

Isaacs syndrome: A review.

Isaacs syndrome is a peripheral nerve hyperexcitability (PNH) syndrome that presents as continuous motor activity. Clinical findings include cramps, fasciculations, and myokymia. Electrodiagnosis plays a key role in diagnosis by demonstrating after-discharges on nerve conduction studies, and fasciculation potentials, myokymic discharges, neuromyotonic discharges, and other types of abnormal spontaneous activity on needle examination. Etiopathogenesis involves the interaction of genetic, autoimmune, and paraneoplastic factors, which requires a broad-ranging evaluation for underlying causes. Initial treatment is symptomatic, but immune therapy is often needed and can be effective. The purpose of this review is to describe the syndrome and its pathogenesis, assist the reader in evaluating patients with suspected Isaacs syndrome and distinguishing it from other disorders of PNH, and suggest an approach to management, including both symptomatic and immunomodulating therapy.

Operational Impact of Neurology Rural Access Model: Reflections on the Importance of Demand-Shaping.

Background and Objectives: Demand for specialty neurologic care has been steadily increasing over the past several decades, and health systems are needing to meet the demands of their patients while managing a dwindling workforce. This retrospective study investigates the operational impact of a regional neurology clinic staffed by advanced practice providers with remote physician oversight in a "hub and spoke" delivery model to serve lower complexity patients. Methods: A retrospective, cross-sectional study was conducted to evaluate outcomes. Descriptive analysis of referral volumes, cancellation/no-show rates, and patient complexity as determined by the reason for referral were used to evaluate patients who received referrals from rural counties north of the primary neurology practice before and after the opening of the regional clinic. These metrics were evaluated longitudinally from counties of interest and for differences in patients seen at the regional clinic vs primary neurology practice. Results: Referral volumes from the northern counties increased at significantly higher rates after the opening of the regional clinic than other counties in the serviced area. This resulted in an increase in patients seen in the hub clinics and spoke clinic. The regional clinic did see patients who were less complex than the primary practice; however, the total volume of low-complexity patients scheduled at the primary practice did not decrease. Cancellation and no-show rates did not seem to be affected in either clinic. Discussion: The opening of a regional "spoke" clinic resulted in the generation of greater referral volumes that exceeded the capacity created by the clinic. Owing to this, there was an increase in the number of patients seen from the regional counties in the hub clinics, negating the potential benefit of improving access for high-complexity patients. Importance of demand-shaping and appropriate utilization as part of the value equation are discussed, followed by discussion of mitigation strategies.

Gold Carding Policies: Reducing the Barriers Between Payers and Providers.

A central approach to achieving high-quality neurologic care is to reduce the burden on providers in accessing services needed to achieve this level of care. Neurology-based practices across the continuum (solo, multispecialty, hospital, or health system-based) have adopted different methods to mitigate the impact of gatekeeper methods of prior authorization and related mechanisms. We discuss ways to partner with payers through innovative Gold Carding programs that reduce the burden of gatekeeper mechanisms on neurology providers, thereby allowing them to consistently focus their efforts in the provision of high-quality neurologic care.

Implementation of E-Consults in an Integrated Health System for a Value Population.

The adoption of e-consults, a form of formal, asynchronous provider-to-provider communication in which specialty medical advice is sought, has been proven to reduce unnecessary specialty consultations, build provider relationships, and reduce fragmentation of care. While the utilization of e-consults is generally a well-accepted alternative method of incorporating specialist expertise into a patient's plan of care and adoption has become common, the implementation of this disruptive care delivery modality can be challenging. This article seeks to describe the process and operational outcomes of e-consult adoption in an integrated health system with a focus on the benefits in the context of value-based care. Implications of e-consult adoption on referral volumes, wait times, and cancellation rates at the service line level are investigated. E-consult adoption and completion metrics, including utilization, completed versus rejected, turn-around times, and projected cost savings, are also discussed.

A Shortage of Neurologists We Must Act Now: A Report From the AAN 2019 Transforming Leaders Program.

In nearly every US state today, a large mismatch exists between the need for neurologists and neurological services and the availability of neurologists to provide these services. Patients with neurologic disorders are rising in prevalence and require access to high level care to reduce disability. The current neurology mismatch reduces access to care, worsens patient outcomes, and erodes career satisfaction and quality of life for neurologists as they face increasingly insurmountable demands. As a community, we must address this mismatch in the demand and supply of neurological care in an aggressive and sustained manner to ensure the future health of our patients and our specialty. The American Academy of Neurology has multiple ongoing initiatives to help reduce and resolve the existing mismatch. With the intent of raising awareness and widening the debate nationally, we present a strategic plan that the Academy could implement to coordinate and expand existing efforts. We characterize the suggested strategies as: shaping the demand, enhancing the workforce, and advocating for neurologist value The proposed framework is based on available data and expert opinion when data were lacking. Prioritization of strategies will vary by geography, practice setting, and local resources. We believe the time to act is now, to allow concerted effort and targeted interventions to avert this looming public health crisis.

Patient Assisted Intervention for Neuropathy: Comparison of Treatment in Real Life Situations (PAIN-CONTRoLS): Bayesian Adaptive Comparative Effectiveness Randomized Trial.

Importance: Cryptogenic sensory polyneuropathy (CSPN) is a common generalized slowly progressive neuropathy, second in prevalence only to diabetic neuropathy. Most patients with CSPN have significant pain. Many medications have been tried for pain reduction in CSPN, including antiepileptics, antidepressants, and sodium channel blockers. There are no comparative studies that identify the most effective medication for pain reduction in CSPN. Objective: To determine which medication (pregabalin, duloxetine, nortriptyline, or mexiletine) is most effective for reducing neuropathic pain and best tolerated in patients with CSPN. Design, Setting, and Participants: From December 1, 2014, through October 20, 2017, a bayesian adaptive, open-label randomized clinical comparative effectiveness study of pain in 402 participants with CSPN was conducted at 40 neurology care clinics. The trial included response adaptive randomization. Participants were patients with CSPN who were 30 years or older, with a pain score of 4 or greater on a numerical rating scale (range, 0-10, with higher scores indicating a higher level of pain). Participant allocation to 1 of 4 drug groups used the utility function and treatment's sample size for response adaptation randomization. At each interim analysis, a decision was made to continue enrolling (up to 400 participants) or stop the whole trial for success (80% power). Patient engagement was maintained throughout the trial, which helped guide the study and identify ways to communicate and disseminate information. Analysis was performed from December 11, 2015, to January 19, 2018. Interventions: Participants were randomized to receive nortriptyline (n = 134), duloxetine (n = 126), pregabalin (n = 73), or mexiletine (n = 69). Main Outcomes and Measures: The primary outcome was a utility function that was a composite of the efficacy (participant reported pain reduction of ≥50% from baseline to week 12) and quit (participants who discontinued medication) rates. Results: Among the 402 participants (213 men [53.0%]; mean [SD] age, 60.1 [13.4] years; 343 White [85.3%]), the utility function of nortriptyline was 0.81 (95% bayesian credible interval [CrI], 0.69-0.93; 34 of 134 [25.4%] efficacious; and 51 of 134 [38.1%] quit), of duloxetine was 0.80 (95% CrI, 0.68-0.92; 29 of 126 [23.0%] efficacious; and 47 of 126 [37.3%] quit), pregabalin was 0.69 (95% CrI, 0.55-0.84; 11 of 73 [15.1%] efficacious; and 31 of 73 [42.5%] quit), and mexiletine was 0.58 (95% CrI, 0.42-0.75; 14 of 69 [20.3%] efficacious; and 40 of 69 [58.0%] quit). The probability each medication yielded the highest utility was 0.52 for nortriptyline, 0.43 for duloxetine, 0.05 for pregabalin, and 0.00 for mexiletine. Conclusions and Relevance: This study found that, although there was no clearly superior medication, nortriptyline and duloxetine outperformed pregabalin and mexiletine when pain reduction and undesirable adverse effects are combined to a single end point. Trial Registration: ClinicalTrials.gov Identifier: NCT02260388.

Current Treatment Options for Peripheral Nerve Hyperexcitability Syndromes.

PURPOSE OF REVIEW: Peripheral nerve hyperexcitability (PNH) syndromes are divided into primary and secondary groups based on the presence or absence of demonstrable peripheral nerve disease. In this review, we systematically evaluate the evidence for current therapies and supportive managements based on autoimmune, paraneoplastic, and genetic components in pathophysiology reported in the literature. RECENT FINDINGS: Current therapy options are based on symptomatic management as well as focusing the underlying immune/genetic/paraneoplastic pathology by immunosuppressants, chemotherapy, and surgery. Further research is desired to provide treatment options geared specifically towards addressing PNH. Supportive care can also be an area for future research.

Pyridoxine Toxicity Small Fiber Neuropathy With Dysautonomia: A Case Report.

Pyridoxine (vitamin B6) toxicity is a well-known cause of primary sensory, length-dependent, axonal polyneuropathy. Although sensory symptoms predominate, autonomic symptoms have also been reported in some cases. To date, there is no objective evidence of autonomic dysfunction reported in the literature. We present the case of a 41-year-old woman with 2 years of progressive burning pain, numbness, tingling, and weakness in a stocking-glove distribution who was found to have severe pyridoxine toxicity. Concurrent presence of large and small fiber nerve dysfunction was noted in the form of abnormal electromyography/nerve conduction study demonstrating a chronic sensory polyneuropathy and autonomic testing demonstrating abnormal responses to quantitative sweat testing and cardiovagal function testing. This case highlights the need for consideration of small fiber nerve damage by obtaining autonomic testing in cases of pyridoxine toxicity.

Advanced Practice Clinician Training for Neurology.

BACKGROUND: The specialty of Neurology is faced with a fundamental problem of economics: supply and demand. The projected increase in provider supply is unlikely to keep up with projected increases in patient-care demand. Many large academic centers have used residents to meet this patient-care demand. However, the conflict between education of residents and patient-care needs has created a hindrance to both of those missions. Many specialties have been using advanced practice clinicians (APCs) to help address the need for patient care. In the setting of a residency program, this availability of APCs can help to alleviate patient-care demands for the resident and allow for better allocated educational time. Neurology has not historically been a popular choice for APCs and a standardized educational curriculum for a Neurology APC has not been established. METHODS: The authors share an example curriculum recently implemented for training new inpatient Neurology APCs. This curriculum includes a 12-week program complete with rotations through various subspecialties and proposes fundamental lecture topics for use in education. The authors share their expectations for clinical duties that evolve over the course of the 12-week program in conjunction with expectations for increasing clinical knowledge as well as efficiency in system utilization. CONCLUSION: The addition of APCs to support a busy inpatient Neurology practice has obvious beneficial implications but the integration and education of this new staff must be structured and well-designed to support the confidence of the APC in both their knowledge and their role as an indispensable member of the care team.

Pseudobulbar affect: prevalence and management.

Pseudobulbar affect (PBA) may occur in association with a variety of neurological diseases, and so may be encountered in the setting of amyotrophic lateral sclerosis, extrapyramidal and cerebellar disorders, multiple sclerosis, traumatic brain injury, Alzheimer's disease, stroke, and brain tumors. The psychological consequences and the impact on social interactions may be substantial. Although it is most commonly misidentified as a mood disorder, particularly depression or a bipolar disorder, there are characteristic features that can be recognized clinically or assessed by validated scales, resulting in accurate identification of PBA, and thus permitting proper management and treatment. Mechanistically, PBA is a disinhibition syndrome in which pathways involving serotonin and glutamate are disrupted. This knowledge has permitted effective treatment for many years with antidepressants, particularly tricyclic antidepressants and selective serotonin reuptake inhibitors. A recent therapeutic breakthrough occurred with the approval by the Food and Drug Administration of a dextromethorphan/quinidine combination as being safe and effective for treatment of PBA. Side effect profiles and contraindications differ for the various treatment options, and the clinician must be familiar with these when choosing the best therapy for an individual, particularly elderly patients and those with multiple comorbidities and concomitant medications.

Rehabilitation of brachial plexus and peripheral nerve disorders.

Peripheral nerve lesions are common and can present in a variety of ways. Peripheral nerve injury can result from a broad spectrum of causes. For the majority of patients, rehabilitation is generally indicated regardless of etiology. Evaluation and treatment by a multidisciplinary team including neurologists, psychiatrists, surgeons, occupational and physical therapists, and therapists with specialized training in orthotics maximizes the potential for recovery. This chapter will focus on those upper and lower extremity neuropathies that are most commonly seen in clinical practice. In addition, we discuss various rehabilitative strategies designed to improve function and quality of life.

Amyotrophic lateral sclerosis: what role does environment play?

The cause of sporadic amyotrophic lateral sclerosis (ALS) is not known. Studies associate toxic, dietary, infectious, neoplastic, and physical factors as underlying, predisposing, or pathogenic influences. Historical, conventional, and novel disease mechanisms, acting solely or in concert, convert previously healthy individuals into terminally ill patients. Despite intensive investigations in the previous decades, the underlying cause and effective treatments elude researchers. Discovering causative mechanisms in sporadic ALS will facilitate effective treatments and cures for this disorder. After a brief review of the disease process itself, this article discusses potential environmental influences on the development of ALS.

Meralgia paresthetica and femoral acetabular impingement: a possible association.

UNLABELLED: Meralgia paresthetica consists of pain and dysesthesia in the anterolateral thigh. Etiology is divided into spontaneous and iatrogenic causes. To my knowledge this has never been attributed to femoral acetabular impingement. This case highlights the presence of lateral femoral cutaneous neuropathy in the setting of femoral acetabular impingement syndrome thus raising the possibility of an association. KEYWORDS: Femoral acetabular impingement; Lateral femoral cutaneous nerve; Dysesthesia; Nerve conduction studies.

Recovery of neurologic dysfunction with early intervention of vitamin B12.

A patient developed numbness and tingling in distal extremities with subsequent weakness. Evaluation revealed B12 deficiency. She had evidence of myelopathy on imaging studies and polyneuropathy on electrodiagnostic testing. Treatment with B12 caused remittance of symptoms and resolution/improvement of abnormalities found on the imaging and electrodiagnostic studies. This case demonstrates that early intervention with B12 supplementation can cause reversal of both central and peripheral nervous system dysfunction.