Interstitial amyloidosis in sporadic inclusion body myositis.

INTRODUCTION/AIMS: Intracellular congophilic inclusions within muscle fibers, although nonspecific, are one of the pathological hallmarks of sporadic inclusion body myositis (sIBM). Extracellular amyloid deposits in muscle, on the other hand, are the canonical findings of amyloid myopathies, which occur with or without systemic amyloidosis. METHODS: We reviewed the muscle biopsy database (1998-2020) to identify sIBM patients with extracellular amyloid deposits. Clinical and laboratory data were reviewed. RESULTS: We identified five sIBM patients (three clinicopathologically defined and two clinically defined) with extracellular amyloid deposits in muscle. Mean age at diagnosis was 74.8 y (range, 68-84 y). All patients had a typical sIBM pattern of weakness without associated sensory or autonomic symptoms. None had electrophysiological evidence of peripheral neuropathy. Only one patient had a monoclonal gammopathy (immunoglobulin M-lambda, IgM-λ) with normal bone marrow biopsy. This patient with monoclonal gammopathy and three other patients underwent abdominal fat pad aspirate and were negative for amyloid. Cardiac evaluation was unrevealing in the four patients tested. Three patients without monoclonal gammopathy had normal transthyretin gene sequencing and inconclusive mass spectrometry-based analysis. The patient with monoclonal gammopathy died of pneumosepsis 5 y after diagnosis and autopsy revealed multi-organ transthyretin amyloidosis. DISCUSSION: Detection of extracellular amyloid deposition in muscle should trigger an aggressive search for systemic amyloidosis independently from other associated myopathological abnormalities. Amyloid subtyping is crucial for early therapy and mortality prevention. An isolated monoclonal gammopathy should not halt a search for non-hematological causes of systemic amyloidosis.

Current Understanding of the Pathophysiology and Approach to Tension-Type Headache.

PURPOSE OF REVIEW: Description of headache dates back thousands of years, and to date, tension-type headache (TTH) remains the most common form of headache. We will review the history and current understanding of the pathophysiology of TTH and discuss the recommended clinical evaluation and management for this syndrome. RECENT FINDINGS: Despite being the most prevalent headache disorder, TTH pathophysiology remains poorly understood. Patients with TTH tend to have muscles that are harder, more tender to palpation, and may have more frequent trigger points of tenderness than patients without headache. However, cause and effect of these muscular findings are unclear. Studies support both peripheral and central mechanisms contributing to the pain of TTH. Diagnosis is based on clinical presentation, while the focus of evaluation is to rule out possible secondary causes of headache. Treatment options have remained similar over the course of the past decade, with some additional studies supportive of both pharmacological and non-pharmacological options. An approach to TTH has been outlined including historical context, evolution over time, and the best evidence regarding our current understanding of the complex pathophysiology and treatment of this disease.

First Bite Syndrome: What Neurologists Need to Know.

PURPOSE OF REVIEW: Though first bite syndrome is well known in surgical settings, it is not commonly included in the differential for sharp paroxysmal facial pain in the neurology literature. This paper will highlight the clinical features and relevant anatomy of first bite syndrome, with the goal of helping clinicians differentiate this from other similar facial pain disorders. RECENT FINDINGS: First bite syndrome is severe sharp or cramping pain in the parotid region occurring with the first bite of each meal and improving with subsequent bites. Pathophysiology has been attributed to imbalanced sympathetic/parasympathetic innervation of the parotid gland. This is seen most typically in the post-surgical setting following surgery in the parotid or parapharyngeal region, but neoplastic etiologies have also been reported. It is common for patients to present with concurrent great auricular neuropathy and/or Horner's syndrome. Evidence regarding treatment is limited to case reports/series, however, botulinum toxin injections and neuropathic medicines have been helpful in select cases. It is critical for clinicians to be able to differentiate first bite syndrome from other paroxysmal facial pain. To help with this, we have proposed diagnostic criteria for clinical assessment. Patients often improve gradually over time, but symptomatic treatment with botulinum toxin or neuropathic medicine may be required.

5-HT1F agonists.

5-Hydroxytryptamine (HT)/serotonin receptor agonism has been a long-recognized property of triptan medications, and more recently, the study and development of medications with selective binding to the 1F receptor subtype have been explored. While the exact mechanism contributing to decreased symptoms of an acute migraine attack remains unclear, selective 5-HT1F agonists have demonstrated clinical efficacy with lasmiditan as the only approved medication from this class to date. Lasmiditan lacks vasoconstrictive properties, giving it utility in specific patient populations in whom triptans should be avoided. Availability, central nervous system (CNS) side effects, and 8-hour driving restriction may affect its clinical use.

Improving Neurology Inpatient Fall Rate: Effect of a Collaborative Interdisciplinary Quality Improvement Initiative.

Objective: To reduce unwitnessed inpatient falls on the neurology services floor at an academic medical center by 20% over 15 months. Patients and Methods: A 9-item preintervention survey was administered to neurology nurses, resident physicians, and support staff. Based on survey data, interventions targeting fall prevention were implemented. Providers were educated during monthly in-person training sessions regarding the use of patient bed/chair alarms. Safety checklists were posted inside each patient's room reminding staff to ensure that bed/chair alarms were on, call lights and personal items were within reach, and patients' restroom needs were addressed. Preimplementation (January 1, 2020, to March 31, 2021) and postimplementation (April 1, 2021, to June 31, 2022) rates of falls in the neurology inpatient unit were recorded. Adult patients hospitalized in 4 other medical inpatient units not receiving the intervention served as a control group. Results: Rates of falls, unwitnessed falls, and falls with injury all decreased after intervention in the neurology unit, with rates of unwitnessed falls decreasing by 44% (2.74 unwitnessed falls per 1000 patient-days before intervention to 1.53 unwitnessed falls per 1000 patient-days after intervention; P=.04). Preintervention survey data revealed a need for education and reminders on inpatient fall prevention best practices given a lack of knowledge on how to operate fall prevention devices, driving the implemented intervention. All staff reported significant improvement in operating patient bed/chair alarms after intervention (P<.001). Conclusion: A collaborative, multidisciplinary approach focusing on provider fall prevention education and staff checklists is a potential technique to reduce neurology inpatient fall rates.