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Neurologic Emergencies and Neurocritical Care

Clinical Vignette A 62-year-old man with hypertension and diabetes mellitus type 2 was brought to the emergency department via Emergency Medical Services for evaluation of acute onset of obtundation. Over the course of approximately 1 minute, he changed from being fully alert and talking normally to minimally responsive. En route, his vital signs were blood pressure 156/82 mm Hg, pulse 92 beats/minute, oxygen saturation 95% on 2 L…

Cranial Nerves XI and XII: Accessory and Hypoglossal

Cranial Nerve XI: the Spinal Accessory Nerve Cranial nerve (CN) XI, or the spinal accessory nerve (SAN), serves primarily as the motor nerve for the sternocleidomastoid (SCM) and trapezius muscles in the neck and shoulder. It has an intriguing functional array with one of the two major muscles it innervates, the SCM, inserting on the ipsilateral occiput. When one side contracts, it turns the head in…

Cranial Nerves IX and X: Glossopharyngeal and Vagus

Cranial Nerve IX: Glossopharyngeal Nerve and Swallowing The glossopharyngeal nerve is a mixed nerve, containing both sensory and motor fibers along with parasympathetic, special sensory, and visceral sensory components. The motor component is fibers to the stylopharyngeus muscle, as well as the superior pharyngeal constrictors, and the sensory component has a similar distribution over the upper pharynx and posterior of the tongue, as well as the…

Cranial Nerve VIII: Auditory and Vestibular

Auditory Nerve Clinical Vignette A 68-year-old man presented with sudden onset of unilateral right-sided hearing loss. He stated that this was preceded by several months of constant ringing in his right ear. He had a history of hypertension and type 2 diabetes mellitus that was well controlled with oral hypoglycemic agents. He had no recent head trauma or previous head or neck surgeries. His only medications…

Cranial Nerve VII: Facial

Clinical Vignette A 62-year-old judge became aware of subtle weakness of his left lower face that he first noted while shaving. Two months later, he noted that he could no longer close his left eyelid fully and was having increasing weakness of the remainder of his left face. He was referred to a neurologist, who reassured him that he had a “benign” Bell palsy. He sought…

Cranial Nerve V: Trigeminal

Clinical Vignette A 58-year-old retired town clerk presented with a 2-week history of numbness over her left chin and adjacent lower lip as well as vague pain in the left jaw. She explained that the area feels “Novacaine-like,” exactly like the sensation she had experienced numerous times recently because of extensive “dental work” requiring mandibular blocks. She reported a 5-year history of lichen planus of her…

Cranial Nerves III, IV, and VI: Oculomotor, Trochlear, and Abducens Nerves: Ocular Mobility and Pupils

Cranial Nerve III: Oculomotor Clinical Vignette A 37-year-old woman presented with a 2-day history of “blurry” vision on upward gaze and headache. One month previously, when she had experienced the same symptoms, sinusitis was diagnosed, and an antibiotic was prescribed; symptoms had resolved in 5 days. Examination demonstrated impaired upward, downward, and medial movement in the right eye. There was mild right-sided ptosis, and the right…

Cranial Nerve II: Optic Nerve and Visual System

Intraocular Optic Nerve Clinical Vignette A 48-year-old man was referred for sudden loss of vision in the left eye. He had noted that morning while shaving that he could not see the lower half of his chin with the right eye closed. He had no pain and had no preceding systemic symptoms. His past medical history was noteworthy for mild diet-controlled hypercholesterolemia and untreated labile hypertension.…

Cranial Nerve I: Olfactory

Clinical Vignette A 64-year-old woman, a retired music teacher and a food and wine connoisseur, was driving to the airport to catch a flight to Spain when she noted she could not smell the characteristic skunk odor her friend was complaining about. She thought it had something to do with her recent cold. While traveling in Spain, she gradually became more alarmed as she realized that…

Neuroimaging in Neurologic Disorders

The armamentarium of neuroimaging techniques includes: computed tomography (CT), magnetic resonance imaging (MRI), ultrasound, x-ray, fluoroscopy, and nuclear medicine (NM). Of these, CT and MRI have found the most use in daily practice and are frequently used as first-line neuroimaging, with the remaining modalities providing important, often ancillary, functions. The strength of CT lies in its high spatial resolution, speed of acquisition, and lack of required…

Laboratory Testing in Neurology

Clinical Vignette A 45-year-old woman presents to clinic with episodic left-sided weakness, dysarthria, and gait imbalance. The first episode occurred 9 months ago upon awakening and resolved gradually over 2 days. She had no associated headache, visual symptoms, or nausea. She has had three additional episodes of increasing severity over the following 8 months and has had incomplete recovery following the two most recent episodes. She…

Clinical Neurologic Evaluation

The neurologic sciences are the most intellectually challenging, unequivocally fascinating, and tremendously stimulating of the various clinical disciplines. Initially, the vast intricacies of basic neuroanatomy and neurophysiology often seem overwhelming to both medical student and neuroscience resident alike. However, eventually the various portions of this immense knowledge base come together in a discernible pattern, not unlike a Seurat canvas. Often one is expanding or revisiting our…

Search Strategy for Genetic Movement Disorders

Overview Making a precise genetic, molecular diagnosis provides substantial value to families, even if there is no disease-specific treatment. A specific genetic diagnosis can narrow the horizon of prognostic possibility and help families to identify and network with other similarly affected persons. It can also clarify risks and provide useful information for relatives of the affected individual. Efficient use of online resources is essential for neurologists…

Drug Appendix

Acetazolamide Actions: A weak diuretic and carbonic anhydrase inhibitor. Contraindications: Hyponatremia, hypokalemia, hyperchloremic acidosis, adrenocortical insufficiency, a history of allergy to sulfa drugs (a sulfonamide derivative), and significant renal, or hepatic dysfunction. Main drug interactions: Increases the serum levels of primidone, pseudoephedrine, quinidine, and lithium. Increased risk of nephrolithiasis, especially when used in combination with topiramate. Main side effects: Drowsiness, dizziness, fatigue, paresthesias of extremities and…

Functional Movement Disorders

Introductions A common presenting problem in neurology is the existence of disorders that present with neurological symptoms, but that are atypical for and incongruent with known neurologic disorders. Many different terms have been used to describe these disorders including “hysterical, conversion, psychogenic, dissociative, somatoform, nonorganic, and functional.” These terms reflect the concept that the symptoms are not based on identifiable organic disorders and that the symptoms…

Drug-Induced Movement Disorders in Children

Introduction and Overview Recognizing and managing drug-induced movement disorders (DIMDs) in children poses many challenges. Prescribed medications as well as drugs of abuse can induce a variety of movement disorders including tremor, akathisia, hypokinetic/rigid syndromes (parkinsonism), dystonia, chorea, ataxia, tics, and stereotypies, alone or in combination. In the clinical setting of a new prescription or dose increase, the diagnosis may be relatively straightforward. However, if the…

Movement Disorders and Neuropsychiatric Conditions

Introduction and Overview Caring for children with movement disorders involves substantial exposure to psychiatric diagnoses. This is especially the case in patients with functional (psychogenic) movement disorders, discussed in Chapter 23 . Subthreshold symptoms as well as overt psychiatric diagnoses commonly co-occur with many pediatric movement disorders (as well as myriad other symptomatic and genetic conditions involving cerebral cortex, basal ganglia, and cerebellum). To give just…

Cerebral Palsy

Introduction The term cerebral palsy ( CP ) has been considered by some to be a nonspecific “wastebasket” and by others a valuable diagnostic tool that provides individuals with greater access to medical care, rehabilitation, educational, and social services. The diagnosis of this heterogeneous syndrome rests upon the presence of a permanent motor disability (movement and posture) causing a functional limitation, that is due to a…

Movement Disorders in Sleep

Introduction Sleep is a universal experience; infants spend more than half of their time sleeping and adults about one-third of the day asleep. Sleep is an active process, often accompanied by various movements, some of which are normal, whereas others might have a more pathologic basis. Pediatric neurologists are frequently confronted with interpreting descriptions of movements that occur during sleep. Is the described movement abnormal, a…

Movement Disorders in Autoimmune Diseases

Introduction An autoimmune disorder indicates an acquired process targeting the nervous system involving either autoreactive lymphocytes (T or B cells) or autoantibodies. These disorders are complex, multifactorial entities with potential involvement of underlying genetic vulnerabilities, innate and acquired immune systems, and environmental factors. , In this chapter, autoimmune disorders occurring in children and presenting with associated movement disorders have been divided into five major categories (see…