Autoimmune polyneuropathies.

The autoimmune peripheral neuropathies with prominent motor manifestations are a diverse collection of unusual peripheral neuropathies that are appreciated in vast clinical settings. This chapter highlights the most common immune-mediated, motor predominant neuropathies excluding acute, and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP, respectively). Other acquired demyelinating neuropathies such as distal CIDP and multifocal motor neuropathy will be covered. Additionally, the radiculoplexus neuropathies, resulting from microvasculitis-induced injury to nerve roots, plexuses, and nerves, including diabetic and nondiabetic lumbosacral radiculoplexus neuropathy and neuralgic amyotrophy (i.e., Parsonage-Turner syndrome), will be included. Finally, the motor predominant peripheral neuropathies encountered in association with rheumatological disease, particularly Sjögren's syndrome and rheumatoid arthritis, are covered. Early recognition of these distinct motor predominant autoimmune neuropathies and initiation of immunomodulatory and immunosuppressant treatment likely result in improved outcomes.

Analysis of the spatial distribution of amyotrophic lateral sclerosis in Virginia.

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that is usually fatal. Environmental exposures have been posited in the etiology of ALS, but few studies have modeled the spatial risk of ALS over large geographic areas. In this paper, our goal was to analyze the spatial distribution of ALS in Virginia and identify any areas with significantly elevated risk using Virginia ALS Association administrative data. Methods: We used Bayesian hierarchical spatial regression models to estimate the relative risk for ALS in Virginia census tracts, adjusting for several covariates posited to be associated with the disease. We used an intrinsic conditional autoregressive prior to allow for spatial correlation in the risk estimates and stabilize estimates over space. Results: Considerable variation in ALS risk existed across Virginia, with greater relative risk found in the central and western parts of the state. We identified significantly elevated relative risk in a number of census tracts. In particular, Henrico, Albemarle, and Botetourt counties all contained at least four census tracts with significantly elevated risk. Conclusions: We identified several areas with significantly elevated ALS risk across Virginia census tracts. These results can inform future studies of potential environmental triggers for the disease, whose etiology is still being understood.

Pulmonary Edema and Stunned Myocardium in Subarachnoid Hemorrhage.

Aneurysmal subarachnoid hemorrhage is a life-threatening event that can cause permanent disability. This life-threatening event can be further complicated by subsequent cardiac and pulmonary disability. The presence of a neurogenic cardiomyopathy and pulmonary edema increases the morbidity and mortality of patients who suffer from aneurysmal subarachnoid hemorrhage.  In this paper, we discuss a 39-year-old woman who presented to the emergency department (ED) with a chief complaint of a pounding headache with associated nausea and vomiting for the past three days. She had a past medical history significant only for migraines. During her stay in the ED, she began to exhibit signs of altered consciousness, hemoptysis, and respiratory compromise. Neuroimaging showed evidence of subarachnoid hemorrhage. The exact source of her subarachnoid hemorrhage could not be located with neuroimaging or angiography. Her clinical course was complicated by pulmonary edema and neurogenic stunned myocardium, and is still ongoing.

Hemoptysis in Pediatric Patients.

Hemoptysis is defined as the expectoration of blood or blood-tinged sputum. Blood-tinged sputum is a rare finding in the pediatric population. Finding the cause and treatment of the hemoptysis in pediatric patients is largely dependent on the history. In children, the most common causes of hemoptysis are infection and tracheostomy-related complications. Other causes include aberrant bronchial circulation, aspiration of foreign bodies, and bronchiectasis associated with cystic fibrosis. Due to the rarity of hemoptysis in pediatric patients, diagnosis and management of these patients can be difficult. It is important to refer to case reports and literature to best manage these patients. We report a case of a 3-year-old male patient who presented to the emergency department (ED) with a one-day history of hemoptysis. He presented with his adopted mother who was unable to provide a comprehensive past medical or family history other than stating that the patient has had recurrent bronchial infections since his adoption. She stated that the patient had only one episode of hemoptysis just prior to arrival. The patient did not appear to be in any respiratory distress and did not have any episodes of hemoptysis while in the ED. Due to his afebrile status and lack of evidence of current bleeding, the only intervention administered was an albuterol breathing treatment. He responded well to the breathing treatment and was discharged home with instructions to follow up with his primary care provider.