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Chorea

Clinical Vignette Sylvia is a 59-year-old, right-handed teacher who developed jerky movements in her body within the past 3–4 years and was now “fidgety” all the time. Her family observed intellectual changes; she seemed withdrawn and forgetful, was occasionally inappropriate in her behavior, had to retire early due to inability to “concentrate,” and appearing “nervous” all the time. She had no other past medical history. Her…

Dystonia

Clinical Vignette A 22-year-old previously healthy woman developed slurred speech, difficulty walking, and hand tremors over the course of 1 month after a severe psychologic trauma. She had mild neck pain and took cyclobenzaprine without benefit. She denied any history of fever, head trauma, or exposure to dopamine receptor blocking agents. There was no family history of neurologic disease. Exam revealed lower facial dystonia, including risus…

Tremors

Clinical Vignette A 31-year-old woman had the spontaneous onset of a relatively mild horizontal (“no-no”) head tremor. She had no family history of neurologic disorders. This tremor was initially inconsequential but it gradually increased in severity. Turning her head to the right seemed to increase the tremor, whereas turning her head to the left decreased the tremor. Mild finger pressure on her left chin dampened the…

Atypical Parkinsonian Syndromes

Atypical parkinsonian syndromes, previously called Parkinson-plus syndromes, are chronic, progressive neurodegenerative disorders characterized by rapidly evolving parkinsonism in association with other signs of neurologic dysfunction beyond the spectrum of idiopathic Parkinson disease (PD). These include early dementia, postural instability, supranuclear gaze palsy, early autonomic failure, and pyramidal, cerebellar, or cortical signs. The most common disorders ( Table 29.1 ) are progressive supranuclear palsy (PSP), corticobasal degeneration…

Parkinson Disease

Clinical Vignette This 64-year-old auto mechanic noticed intermittent tremor in his right hand while working. His wife reported he was not swinging his right arm when walking, and she was also observing tremor in his right hand when they were sitting quietly watching movies. The patient himself noted slowness and clumsiness of the right hand when shaving, writing, and typing on the computer or smartphone. Soon…

Dementia: Mild Cognitive Impairment, Alzheimer Disease, Lewy Body Dementia, Frontotemporal Lobar Dementia, Vascular Dementia

The diagnosis and management of dementia in older adults presents major challenges to the clinician and to society at large. The age-related increase in prevalence of dementia combined with increasing life expectancy is expected to result in a worldwide epidemic within the next few decades. Many of the diseases underlying dementia are definitively diagnosed only at autopsy, including the most common cause of dementia, namely Alzheimer…

Delirium and Acute Encephalopathies

Clinical Vignette A 19-year-old woman is brought to the emergency room after she experiences her first ever generalized seizure. An unenhanced brain computed tomography (CT) and basic blood work are normal. She is initially drowsy and disoriented but gradually awakens, becoming increasingly irritable and impulsive. Both the patient and her parents deny that she used alcohol or recreational drugs. She begins pacing the halls and accosting…

Neurocognitive Examination

This chapter is an overview of the examination of higher cortical function as it relates to cortical anatomy and associated functions. Detailed anatomic drawings of the cerebral cortex are provided for one's review while reading this chapter. By the end of the chapter, the reader should gain a basic understanding of cognitive function, anatomical localization, and the neurocognitive examination. Clinical Vignette A 78-year-old, right-handed woman presents…

Sleep Disorders

Primary sleep disorders, such as sleep apnea syndrome, narcolepsy, periodic limb movements (PLMs), and rapid eye movement (REM) behavior disorder, are common and often underdiagnosed. These conditions result in excessive daytime sleepiness (EDS) and/or disrupted sleep, may have a profound effect on quality of life, and may predispose patients to cardiovascular diseases or other comorbidities. Many people are sleep deprived and EDS plays a significant role…

Epilepsy

Epilepsy is generally defined as an illness of recurrent seizures. The prevalence of epilepsy is estimated at 1 in 200 persons. It affects all ages and is generally a chronic problem with significant personal, social, and economic impact, often affecting the ability to hold jobs and drive. Poor epilepsy control and the seizures themselves can lead to significant cognitive and personality changes as well as chronic…

Pain Pathophysiology and Management

Clinical Vignette Tracy, a 65-year-old right-hand–dominant receptionist is referred to orthopedic surgery for right carpal tunnel release following progressively worsening pain and paresthesia in her right hand. Surgery is performed under conscious sedation without complications, and the patient is discharged home that afternoon to follow up with physical therapy for rehabilitation. However, the patient fails to keep her physical therapy appointment and returns to work 4…

Primary and Secondary Headache

Clinical Vignette A 50-year-old woman is referred to a neurologist because of severe headaches. She first developed headaches during adolescence, and they worsened in the setting of menopause. A typical headache is unilateral and localized to the right frontotemporal and periorbital regions. The pain is described as throbbing and pulsating. When severe, her headaches are associated with nausea, vomiting, photophobia, phonophobia, and visual symptoms. Her headaches…

Trauma to the Spine and Spinal Cord

Clinical Vignette After a day of skiing, six teenagers packed into a small hatchback on their way home. Conditions were icy, and the driver lost control of the vehicle near the base of the mountain, rear-ending another vehicle at approximately 30 miles One of the passengers, an 18-year-old man, struck his head on the seat in front of him. When Emergency Medical Services arrived on scene,…

Trauma to the Brain

Traumatic brain injury (TBI) is a worldwide major source of lifelong morbidity and mortality. It is the leading cause of death in North America for individuals between the ages of 1 and 45. The societal cost is substantial, with approximately 2.5 million emergency department visits per year and 3–5 million Americans living with a TBI-related disability. Many of these injuries involve young individuals; contact sports and…

Cerebral Venous Thrombosis

Clinical Vignette A 45-year-old man with a history of bipolar disorder and binges of alcohol abuse gradually developed global headaches that abruptly worsened over a 6-day period. He presented to the emergency room with excruciating headaches, especially while lying flat or after coughing. He described visual blurring and transient visual dimming with straining or getting up rapidly. He was slightly inattentive but had no focal weakness…

Subarachnoid Hemorrhage

Clinical Vignette A 66-year-old woman experienced the near immediate onset of a terrible temporal pain that radiated into her forehead. She almost lost consciousness. She became nauseated, vomited, and felt disoriented. Her family called emergency medical services, who brought her to the emergency department. There, she was noted to be arousable but sleepy and confused, with no focal motor deficit. She had nuchal rigidity and photophobia.…

Intracerebral Hemorrhage

Clinical Vignette A 40-year-old right-handed man with a past medical history significant for hypertension presented to the emergency department (ED) via ambulance with sudden-onset right hemiparesis. He did not take antithrombotic medications. His initial blood pressure was 184/93 mm Hg. The neurologic exam was notable for normal alertness, preserved ability to follow commands with the left limbs, anarthria, normal brainstem reflexes, leftward gaze deviation, and right hemiplegia.…

Ischemic Stroke

While in recent years ischemic stroke has dropped to the fifth most frequent cause of death in the United States, it remains a leading cause of morbidity, mortality, and long-term disability worldwide with a devastating impact on patients, families, and communities. It is well understood that ischemic stroke represents a constellation of etiologies and mechanisms that often present with similar signs and symptoms. Advances in technology…

Anatomic Aspects of Cerebral Circulation

The brain and meninges are supplied by arteries derived from the common carotid artery (CCA) and vertebrobasilar system ( Fig. 14.1 ). The right CCA usually originates from the brachiocephalic trunk, whereas the left CCA originates directly from the aortic arch. Both vertebral arteries (VAs) originate from the subclavian arteries. The morphologic variants of the CCA and VAs usually are not clinically significant. Open full size…

Coma, Vegetative State, and Brain Death

Coma Clinical Vignette A 57-year-old man with coronary artery disease develops severe chest pain and heaviness on exertion, then loses consciousness while working in the garden with his wife. Emergency Medical Services is called immediately and arrives at the scene within minutes. The man is found pale, unresponsive, and flaccid. The systolic blood pressure is low, approximately 70–85 mm Hg, and he is found to be in…