Chronic neurologic disease

Taking a slightly different approach from our usual clinical articles, Dr Maiden calls upon his many years of private practice as a neurologist to share some of the principles he finds fundamental to the thoughtful and compassionate care of patients with chronic neurologic disease. He especially emphasizes the role of the family physician.     

Acute neurologic infections

This article discusses acute bacterial, viral, toxin-mediated, and parasitic neurologic infections, emphasizing those infections that are potentially treatable, are rapidly fatal if untreated, or pose a significant risk of person-to-person transmission.     

Dysimmune neurologic disorders

This article reviews the clinical manifestations, diagnosis, and current therapy of four neurologic diseases currently believed to be autoimmune in etiology: myasthenia gravis, Guillain-Barré syndrome, multiple sclerosis, and Reye's syndrome. Nursing care of these patients is very challenging and crucial to the survival and prevention of further complications. Nursing interventions for the potential problems faced by these individuals are described.     

Pediatric neurologic exam

Although emergency nurses receive education and training in performing comprehensive and rapid assessment, pediatric patients may prove to be challenging due to dynamic growth, development and maturation. If the emergency department (ED) has limited exposure to pediatric patients, performing assessments and prompt interventions may be daunting. Neonates, infants and young children with illness or trauma have unique and often times subtle signs and symptoms that can change rapidly. Although the neurological exam for older children may be similar to that of an adult, there are significant differences based on maturation. The neurologic exam for neonates and infants provides the nurse with even more opportunity to be familiar with developmental differences. Therefore, it is important for ED nurses to become familiar with typical development and early recognition of neurologic insult.     

Geriatric neurologic emergencies

Geriatrics is an important subspecialty within the field of emergency medicine and represents a burgeoning area of practice. The special vulnerability of elderly patients to neurologic disease and injury and the comparative subtlety of clinical presentation mean that physicians should have a lower threshold for laboratory studies, radiologic imaging, consultation, and admission. Transferring appropriate patients to tertiary centers that offer specialized trauma and neurologic and neurosurgical care greatly enhances survival and functional outcomes.     

Pediatric neurologic emergencies

Neuroemergencies are life-threatening situations in which, whatever the cause, common pathologic phenomena result in secondary brain lesions. The goal of critical care management is to stop these self-aggravating processes as soon as possible. Initial resuscitation is devoted to control of the airway and hemodynamic and hydroelectrolytic stabilization. With mass lesions, minimal computed tomographic exploration immediately precedes surgical decompression. Further critical care adapted to the child's needs requires multimodal monitoring. Normoventilation, deep sedation, osmotherapy with mannitol or hypertonic saline solutions, and optimization of mean arterial pressure are the basis of management. A purely pressure-driven approach aimed at controlling cerebral perfusion pressure could be potentially harmful, and associated measurement of blood flow velocity with transcranial Doppler and jugular bulb oxygen saturation monitoring allows an approach to cerebral blood flow and metabolism. Outcome can be improved in dangerous situations such as severe brain injuries, cerebral arteriovenous malformation rupture, status epilepticus, and acute hydrocephalus, provided that emergency management could be applied efficiently.     

Selected neurologic complications of pregnancy

Many neurologic disorders, such as eclampsia, pseudotumor cerebri, stroke, obstetric nerve palsies, subarachnoid hemorrhage, pituitary tumors, and choriocarcinoma, can develop in the pregnant patient. Maternal mortality from eclampsia, which ranges from 0 to 14%, can be due to intracerebral hemorrhage, pulmonary edema, disseminated intravascular coagulation, abruptio placentae, or failure of the liver or kidneys. Associated fetal mortality ranges from 10 to 28% and is directly related to decreased placental perfusion. Pseudotumor cerebri can be associated with serious visual complications; thus, the therapeutic goal is to prevent loss of vision. The risk of stroke in the pregnant patient is 13 times the risk in the nonpregnant patient of the same age. The major causes of stroke in pregnant patients are arterial occlusion and cerebral venous thrombosis. Lumbar disk prolapse is common in pregnant patients, and lumbosacral plexus injuries can occur during labor or delivery. In addition, peripheral nerve compression or entrapment syndromes are thought to be caused by the retention of fluid during pregnancy. The incidence of subarachnoid hemorrhage during pregnancy is 1 in every 10,000 patients, a rate 5 times higher than in nonpregnant women. Because of a proliferation of prolactin-secreting cells, the pituitary gland can enlarge dramatically during pregnancy, a change that can disclose a previously unknown tumor or cause a known pituitary tumor to become symptomatic. The incidence of choriocarcinoma is 1 in 50,000 full-term pregnancies but 1 in 30 molar pregnancies. This malignant tumor has a high rate of cerebral metastatic lesions. In addition to these disorders that develop during pregnancy, the pregnant state can affect numerous preexisting neurologic conditions, including epilepsy, headaches, multiple sclerosis, myasthenia gravis, spinal cord injury, and brain tumors. We discuss advice for patients with such conditions who wish to become pregnant, recommendations for medical and surgical management, and surgical considerations for neurologic complications during pregnancy.     

Systemic complications of neurologic diseases

The systemic complications associated with neurologic disease constitute an extensive topic, because the central nervous system controls many of the functions of the other organ systems in the body and because the brain cannot live in isolation of these systems. The precise mechanisms of many of these systemic alterations are poorly understood, but they appear to depend on the location and the severity of the initial central nervous system pathologic lesion.     

Infant neurologic assessment.

Infant neurologic assessment reflects the ongoing maturation of the central nervous system. Traditional approaches to assessment cannot be used. Key factors are accurate observation and flexibility in obtaining the data. A case example using a 4-month-old infant illustrates specific approaches to assessment.     

Immunotherapies in neurologic disorders

Therapy for autoimmune demyelinating disorders has evolved rapidly over the past 10 years to include traditional immunosuppressants as well as novel biologicals. Antibody-mediated neuromuscular disorders are treated with therapies that acutely modulate pathogenic antibodies or chronically inhibit the humoral immune response. In other inflammatory autoimmune disorders of the peripheral and central nervous system, corticosteroids, often combined with conventional immunosuppression, and immunomodulatory treatments are used. Because autoimmune neurologic disorders are so diverse, evidence from randomized controlled trials is limited for most of the immunotherapies used in neurology. This review provides an overview of the immunotherapies currently used for neurologic disorders.     

Common emergent pediatric neurologic problems

Although there are a variety of neurologic disease processes that the emergency physician should be aware of the most common of these include seizures, closed head injury, headache, and syncope. When one is evaluating a patient who has had a seizure, differentiating between febrile seizures, afebrile seizures, and SE helps to determine the extent of the work-up. Febrile seizures are typically benign, although a diagnosis of meningitis must not be missed. Educating parents regarding the likelihood of future seizures, and precautions to be taken should a subsequent seizure be witnessed, is important. The etiology of a first-time afebrile seizure varies with the patient's age at presentation, and this age-specific differential drives the diagnostic work-up. A follow-up EEG is often indicated, and imaging studies can appropriate on a nonurgent basis. Appropriate management of SE requires a paradigm of escalating pharmacologic therapy, and early consideration of transport for pediatric intensive care services if the seizure cannot be controlled with conventional three-tiered therapy. Closed head injury frequently is seen in the pediatric emergency care setting. The absence of specific clinical criteria to guide the need for imaging makes management of these children more difficult. A thorough history and physical examination is important to uncover risk factors that prompt emergent imaging. Headaches are best approached by assessing the temporal course, associated symptoms, and the presence of persistent neurologic signs. Most patients ultimately are diagnosed with either a tension or migraine headache; however, in those patients with a chronic progressive headache course, an intracranial process must be addressed and pursued with appropriate imaging. Syncope has multiple causes but can generally be categorized as autonomic, cardiac, or noncardiac. Although vasovagal syncope is the most common cause of syncope, vigilance is required to identify those patients with a potentially fatal arrhythmia or with heart disease that predisposes to hypoperfusion. As such, all patients who present with syncope should have an ECG. Additional work-up studies are guided by the results of individual history and physical examination.     

Lyme disease: neurologic manifestations

Lyme disease is a multisystem infectious, inflammatory, and immune-related disorder that follows the bit of an Ixodes dammini tick infected with Borrelia burgdorferi. Subsequent manifestations are protean. This is particularly true concerning the nervous system. It is estimated that 15% of patients with Lyme disease have neurologic complications. It is very possible that nervous system manifestations of Lyme disease are even more common, and these can occur from weeks to many years following the primary infection and can be quite devastating. A high index of suspicion is necessary in diagnosing Lyme-related disorders, because the majority of patients will not recall a tick bite or characteristic rash, and because the subsequent neurologic manifestations are not unlike those seen with many other diseases. With this awareness and appropriate laboratory testing, a presumptive diagnosis can usually be made without significant difficulty. When the nervous system has been affected by Lyme disease, aggressive antibiotic treatment is usually necessary, often using IV preparations. Usually, there will be significant improvement following the use of antibiotics, but there are some patients who will continue to have persistent, probably irreversible, nervous system abnormalities. This is often the case in patients who have had symptoms for long periods of time or who have had multiple recurrences.