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Cerebellar Ataxia

The cerebellum, a unique brain structure with distinctly organized neuronal circuits, is critical for motor and cognitive functions. The cerebellum has dense neuronal connections with almost all regions of the cerebral cortex and brainstem, and can serve as a “hub” to regulate the many brain functions. While cerebellar dysfunction has been implicated in tremor, dystonia, and autism, the prototypical disorder of the cerebellum is cerebellar ataxia,…

Neuro-Otology: Diagnosis and Management of Neuro-Otological Disorders

Dizziness is a term patients use to describe a variety of symptoms including spinning or movement of the environment (vertigo), lightheadedness, presyncope, or imbalance. Patients may also use the term for other sensations such as visual distortion, internal spinning, nonspecific disorientation, and anxiety. Patients may experience dizziness in isolation or with other symptoms. Neurological causes should be considered when other neurological signs and symptoms are present…

Brainstem Syndromes

Other chapters in this book that deal with symptoms emphasize history as the starting point for generating possibilities for the differential diagnosis. This list of diagnostic considerations is then refined during the examination. This chapter calls for a different approach. When the neurologist evaluates a patient with a brainstem disorder, often the most effective method of diagnosis is to organize the differential diagnosis around the objective…

Cranial and Facial Pain

Headache is an exceedingly common symptom that affects virtually everyone at some time in life. It is estimated that nearly half of the world’s adults have an active headache disorder ( ). Headache is one of the most common reasons for outpatient healthcare visits in the United States. Patients with head and/or face pain typically present for medical attention because the discomfort is severe, interferes with…

Disturbances of Smell and Taste

Disorders of smell and taste can jeopardize human safety and produce loss of appetite, unintended weight loss, malnutrition, occupational disability, and reduced quality of life. In a study of 750 patients with chemosensory disturbances, 68% reported altered quality of life, 46% changes in appetite or body weight, and 56% adverse influences on daily living or psychological well-being ( ). In another study of 445 such patients,…

Neuro-ophthalmology: Ocular Motor System

General Principles of Ocular Motor Control I do not know of any kind of work better fitted for correcting loose habits of observation and careless thinking than a study of the ocular motor nerves. John Hughlings Jackson, 1877 This chapter includes an outline of ocular motor anatomy and physiology pertaining to clinical disorders of eye movements and is divided into two broad categories: those disorders that…

Pupillary and Eyelid Abnormalities

Pupillary Abnormalities Pupil Anatomy and Neural Control The size of the pupil is determined by the balance of action between two muscles embedded in the iris: the sphincter pupillae, under parasympathetic control, and the dilator pupillae, under sympathetic control. The sphincter is located circumferentially around the pupil and constricts the pupil. The dilator is situated radially and dilates the pupil. On exposure to light, the pupil…

Neuro-Ophthalmology: Afferent Visual System

Afferent Visual System Anatomy and Physiology From a conceptual standpoint, it is useful to consider vision as having two components: central or macular vision (high acuity, color perception, and light-adapted) and peripheral or ambulatory vision (low acuity, poor color perception, and dark-adapted). Light, refracted by the cornea and lens, is focused on the retina. For the best possible vision, the image of the object of regard…

Neurogenic Dysphagia

The mechanics of swallowing are like those of an elegant wristwatch. On the surface, this appears to be a simple, perhaps even pedestrian process, but it is actually both tremendously complex and remarkably fascinating. Humans swallow approximately 500 times daily ( ). Normally, swallowing occurs unobtrusively and is afforded scant attention. Malfunction can go completely unnoticed for a time; but when it finally becomes manifest, serious—sometimes…

Dysarthria and Apraxia of Speech

Motor Speech Disorders Motor speech disorders are syndromes of abnormal articulation, the motor production of speech, without abnormalities of language. A patient with a motor speech disorder should be able to produce normal expressive language in writing and to comprehend both spoken and written language. If a listener transcribes into print or type the speech of a patient with a motor speech disorder, the text should…

Aphasia and Aphasic Syndromes

Acknowledgment The authors would like to thank Sarah Schneck, MS, CCC-SLP, in the Department of Hearing and Speech Sciences, Vanderbilt University Medical Center, for assistance, especially with the discussion about speech and language therapy. The study of language disorders involves the analysis of that most human of attributes, the ability to communicate through common symbols. Language has provided the foundation of human civilization and learning, and…

Agnosias

Acknowledgment Portions of this chapter appeared in Kirshner, H.S., 2002. Agnosias, in: Behavioral neurology: Practical science of mind and brain. Butterworth-Heinemann, Boston, pp. 137–158. Agnosias are disorders of recognition. The general public is familiar with agnosia from Oliver Sacks’ patient, who not only failed to recognize his wife’s face but also mistook it for a hat. Sigmund Freud originally introduced the term agnosia in 1891 to…

Limb Apraxias and Related Disorders

Apraxia is an inability to correctly perform learned skilled movements. In limb apraxias, there is an inability to correctly execute these movements in an arm or hand owing to neurological dysfunction. Apraxia is essentially a cognitive deficit in motor programming and results in errors either of the spatiotemporal processing of the movements or in the content of the actions. During the course of an apraxia examination,…

Depression and Psychosis in Neurological Practice

The disciplines of behavioral neurology, neuropsychiatry, and geriatric psychiatry are undergoing a scientific renaissance on a global scale ( ). The distinctions between traditional neurological and idiopathic psychiatric conditions are eroding, and the time is ripe to deconstruct the implicit Cartesian dualism that divides the clinical neurosciences—neurology, psychiatry, and neurosurgery. Brain–behavior relationships are bidirectional and should be considered within social and environmental contexts. Patients with neurological…

Behavior and Personality Disturbances

Mental health has become a public health crisis ( ). In the past decade, suicide rates have increased 33%, mental illness has become the group of disorders with the highest years lost to disability (Global Health Data Exchange; ), and depression surpassed all other chronic illnesses as a source of global disability ( ; https://apps.who.int/iris/bitstream/handle/10665/254610/WHO-MSD-MER-2017.2-eng.pdf;jsessionid=87FBBD04685D824264F8EF206FF39FC7?sequence=1 ). It has been suggested that every medical healthcare provider…

Global Developmental Delay and Regression

Developmental delay occurs in approximately 1%–3% of children. Since developmental delay is common, monitoring a child’s development is an essential component of well-child care. Ongoing assessment of the child’s development at each well-child visit creates a pattern of development that is more useful than measuring the discrete milestone achievements at a single visit; therefore, developmental screening should be completed at each well-child visit ( ). Identification…

Intellectual and Memory Impairments

The term intellect designates the totality of the mental or cognitive operations that compose human thought—the higher cortical functions that make up the conscious mind. The intellect and its faculties, the subject matter of human psychology, are the qualities that most separate human beings from other animals. Memory is a specific cognitive function: the storage and retrieval of information. As such, it is the prerequisite for…

Prolonged Comatose States and Brain Death

Consciousness refers to normal wakefulness with awareness of self and the external environment, interactions, and decisions. Explanations and descriptions of consciousness are complex and cross the disciplines of neuroscience, psychology, and philosophy. In medicine, the assessment of consciousness is a clinical assessment done by observing a patient’s alertness, willful interaction to stimuli, and thought content as expressed by language. Consciousness implies there is the possibility of…

Stupor and Coma

Definitions Consciousness may be defined as a state of awareness of self and surroundings. Alterations in consciousness are conceptualized into two types. The first type affects arousal and is the subject of this chapter. Sleep, the only normal form of altered consciousness, is discussed in Chapter 101 . The second type involves cognitive and affective mental function, sometimes referred to as the “content” of mental function.…

Delirium

Delirium is an acute mental status change characterized by abnormal and fluctuating attention. There is a disturbance in level of awareness and reduced ability to direct, focus, sustain, and shift attention ( ). These difficulties additionally impair other areas of cognition. The syndrome of delirium can be a physiological consequence of a medical condition or stem from a primary neurological cause. Delirium is by far the…