Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease the space for the lower brain stem and cervical cord. These abnormalities can result in neck pain; syringomyelia; cerebellar, lower cranial nerve, and spinal cord deficits; and vertebrobasilar ischemia. Diagnosis is by MRI or […]

Glossopharyngeal neuralgia is characterized by recurrent attacks of severe pain in the 9th and 10th cranial nerve distribution (posterior pharynx, tonsils, back of the tongue, middle ear, under the angle of the jaw). Diagnosis is clinical. Treatment is usually with carbamazepine or gabapentin. Glossopharyngeal neuralgia sometimes results from nerve compression by an aberrant, pulsating artery […]

Facial nerve (7th cranial nerve) palsy is often idiopathic (formerly called Bell palsy). Idiopathic facial nerve palsy is sudden, unilateral peripheral facial nerve palsy. Symptoms of facial nerve palsy are hemifacial paresis of the upper and lower face. Tests (eg, chest x-ray, serum ACE level) are done to diagnose treatable causes. Treatment may include lubrication […]

Hemifacial spasm refers to unilateral painless, synchronous contractions of facial muscles due to dysfunction of the 7th cranial (facial) nerve and/or its motor nucleus. Hemifacial spasm results from nerve compression by a pulsating blood vessel, similar to that in trigeminal neuralgia. The pulsating blood vessel is often visible on MRI, but diagnosis is ultimately clinical. […]

Trigeminal neuralgia is severe paroxysmal, lancinating facial pain due to a disorder of the 5th cranial nerve. Diagnosis is clinical. Treatment is usually with carbamazepine or gabapentin; sometimes surgery is required. Trigeminal neuralgia affects mainly adults, especially the elderly. Etiology Trigeminal neuralgia is usually caused by an intracranial artery (eg, anterior inferior cerebellar artery, ectatic […]

Sixth cranial nerve palsy affects the lateral rectus muscle, impairing eye abduction. The eye may be slightly adducted when the patient looks straight ahead. The palsy may be secondary to nerve infarction, Wernicke encephalopathy, trauma, infection, or increased intracranial pressure, or it may be idiopathic. Determining the cause requires MRI and often lumbar puncture and […]

Fourth cranial nerve palsy impairs the superior oblique muscle, causing paresis of vertical gaze, mainly in adduction. Fourth cranial (trochlear) nerve palsy is often idiopathic. Few causes have been identified. Causes include closed head injury (common), which may cause unilateral or bilateral palsies, and infarction due to small-vessel disease (eg, in diabetes). Rarely, this palsy […]

Third cranial nerve disorders can impair ocular motility, pupillary function, or both. Symptoms and signs include diplopia, ptosis, and paresis of eye adduction and of upward and downward gaze. If the pupil is affected, it is dilated, and light reflexes are impaired. If the pupil is affected or patients are increasingly unresponsive, CT is done […]

Internuclear ophthalmoplegia is characterized by paresis of eye adduction in horizontal gaze but not in convergence. It can be unilateral or bilateral. During horizontal gaze, the medial longitudinal fasciculus (MLF) on each side of the brain stem enables abduction of one eye to be coordinated with adduction of the other. The MLF connects the following […]

A conjugate gaze palsy is inability to move both eyes in a single horizontal (most commonly) or vertical direction. Gaze palsies most commonly affect horizontal gaze; some affect upward gaze, and fewer affect downward gaze. Horizontal gaze palsies: Conjugate horizontal gaze is controlled by neural input from the cerebral hemispheres, cerebellum, vestibular nuclei, and neck. […]

Dysfunction of certain cranial nerves may affect the eye, pupil, optic nerve, or extraocular muscles and their nerves; thus, they can be considered cranial nerve disorders, neuro-ophthalmologic disorders, or both. Neuro-ophthalmologic disorders may also involve dysfunction of the central pathways that control and integrate ocular movement and vision. Cranial nerve disorders can also involve dysfunction […]

Meningeal inflammation that lasts > 2 wk (subacute meningitis) or > 1 mo (chronic meningitis) may have infectious or noninfectious causes (eg, cancer). Diagnosis requires CSF analysis, usually after CT or MRI. Treatment is directed at the cause. Etiology Subacute or chronic meningitis may have infectious or noninfectious causes and may be an aseptic meningitis […]

For brain infections, see Brain Infections, for neonatal meningitis, see Infections in Neonates: Neonatal Bacterial Meningitis ) Meningitis is inflammation of the meninges of the brain or spinal cord. Meningitis is often infectious and is one of the most common CNS infections. Inflammation involves both the meninges and brain parenchyma (meningoencephalitis). Meningitis may become evident […]

The cerebrum is divided by a longitudinal fissure into 2 hemispheres, each containing 5 discrete lobes. The frontal, temporal, parietal, and occipital lobes cover the brain’s surface; the insula is hidden under the Sylvian fissure (see Fig. 1: Function and Dysfunction of the Cerebral Lobes: Areas of the brain.). Although specific functions are attributed to […]

Neuropathic Pain Share This view related topics in this manual Neuropathic pain results from damage to or dysfunction of the peripheral or central nervous system, rather than stimulation of pain receptors. Diagnosis is suggested by pain out of proportion to tissue injury, dysesthesia (eg, burning, tingling), and signs of nerve injury detected during neurologic examination. […]

Chronic pain is pain that persists or recurs for > 3 mo, persists > 1 mo after resolution of an acute tissue injury, or accompanies a nonhealing lesion. Causes include chronic disorders (eg, cancer, arthritis, diabetes) and injuries (eg, herniated disk, torn ligament), and many primary pain disorders (eg, neuropathic pain, fibromyalgia, chronic headache). Various […]

Nonopioid and opioid analgesics are the main drugs used to treat pain. Antidepressants, anticonvulsants, and other CNS-active drugs may also be used for chronic or neuropathic pain and are first-line therapy for some conditions. Neuraxial infusion, nerve stimulation, injection therapies, and neural blockade can help selected patients. Cognitive-behavioral interventions (eg, incremental gains in function; changes […]

Clinicians should evaluate the cause, severity, and nature of the pain and its effect on activities and psychologic well-being. Evaluation of the cause of acute pain (eg, back pain, chest pain—see elsewhere in The Manual) differs from that of chronic pain (see Pain: Symptoms and Signs). The history should include the following information about the […]

Overview of Pain Share This Pain is the most common reason patients seek medical care. Pain has sensory and emotional components and is often classified as acute or chronic. Acute pain is frequently associated with anxiety and hyperactivity of the sympathetic nervous system (eg, tachycardia, increased respiratory rate and BP, diaphoresis, dilated pupils). Chronic pain […]

Pure autonomic failure results from neuronal loss in autonomic ganglia, causing orthostatic hypotension and other autonomic symptoms. Pure autonomic failure, previously called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome, denotes generalized autonomic failure without CNS involvement. This disorder differs from multiple system atrophy because it lacks central or preganglionic involvement. Pure autonomic failure affects more women, […]

Multiple System Atrophy view related topics in this manual Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. It includes 3 disorders previously thought to be distinct: olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome. Symptoms include hypotension, urinary retention, constipation, ataxia, rigidity, and postural instability. Diagnosis is clinical. Treatment […]

Horner’s Syndrome Horner’s syndrome is ptosis, miosis, and anhidrosis due to dysfunction of cervical sympathetic output. Etiology Horner’s syndrome results when the cervical sympathetic pathway running from the hypothalamus to the eye is disrupted. The causative lesion may be primary (including congenital) or secondary to another disorder. Lesions are usually divided into Central (eg, brain […]

Autonomic Neuropathies Share This view related topics in this manual Autonomic neuropathies are peripheral nerve disorders with disproportionate involvement of autonomic fibers. The best known autonomic neuropathies are those accompanying peripheral neuropathy due to diabetes, amyloidosis, or autoimmune disorders. Autoimmune autonomic neuropathy is an idiopathic disorder that often develops after a viral infection; onset may […]

Overview of the Autonomic Nervous System Share This The autonomic nervous system (ANS) regulates physiologic processes. Regulation occurs without conscious control, ie, autonomously. The 2 major divisions are the sympathetic and parasympathetic systems. Disorders of the ANS cause autonomic insufficiency or failure and can affect any system of the body. Anatomy The ANS receives input […]

Overview of Pain Share This Pain is the most common reason patients seek medical care. Pain has sensory and emotional components and is often classified as acute or chronic. Acute pain is frequently associated with anxiety and hyperactivity of the sympathetic nervous system (eg, tachycardia, increased respiratory rate and BP, diaphoresis, dilated pupils). Chronic pain […]

Fatal insomnia is a typically hereditary prion disorder causing difficulty sleeping, motor dysfunction, and death. FI, a very rare disease, usually results from an autosomal dominant mutation, but several sporadic cases have been identified. Average age at onset is 40 yr (ranging from the late 30s to the early 60s). Common early symptoms include difficulty […]

Creutzfeldt-Jakob Disease (CJD) Share This Creutzfeldt-Jakob disease is a sporadic or familial prion disease. Bovine spongiform encephalopathy (mad cow disease) is a variant form. Symptoms include dementia, myoclonus, and other CNS deficits; death occurs in 1 to 2 yr. Transmission can be prevented by taking precautions when handling infected tissues and using bleach to clean […]

Overview of Prion Diseases (Transmissible Spongiform Encephalopathies) Share This Prion diseases are progressive, fatal, and untreatable degenerative brain disorders. They include Creutzfeldt-Jakob disease (CJD), the prototypic example Gerstmann-Sträussler-Scheinker disease (GSS) Fatal insomnia (FI) Variant CJD (vCJD) Kuru Prion diseases usually occur sporadically, with a worldwide annual incidence of about 1/1 million. Prion diseases result from […]

Progressive supranuclear palsy is a rare, degenerative CNS disorder causing loss of voluntary eye movements, bradykinesia, muscular rigidity with progressive axial dystonia, pseudobulbar palsy, and dementia. The cause of progressive supranuclear palsy is unknown. Neurons in the basal ganglia and brain stem degenerate; neurofibrillary tangles containing an abnormally phosphorylated tau protein are also present. Multiple […]

Apraxia is inability to execute purposeful, previously learned motor tasks, despite physical ability and willingness, as a result of brain damage. Diagnosis is clinical, often including neuropsychologic testing, with brain imaging (eg, CT, MRI) to identify cause. Prognosis depends on the cause and extent of damage and patient age. There is no specific treatment, but […]

Aphasia is language dysfunction that may involve impaired comprehension or expression of words or nonverbal equivalents of words. It results from dysfunction of the language centers in the cerebral cortex and basal ganglia or of the white matter pathways that connect them. Diagnosis is clinical, often including neuropsychologic testing, with brain imaging (CT, MRI) to […]

Amnesia is partial or total inability to recall past experiences. It may result from traumatic brain injury, degeneration, metabolic disorders, seizure disorders, or psychologic disturbances. Diagnosis is clinical but often includes neuropsychologic testing and brain imaging (eg, CT, MRI). Treatment is directed at the cause. Processing of memories involves registration (taking in new information), encoding […]

Intracranial tumors may involve the brain or other structures (eg, cranial nerves, meninges). The tumors usually develop during early or middle adulthood but may develop at any age; they are becoming more common among the elderly. Brain tumors are found in about 2% of routine autopsies. Some tumors are benign, but because the cranial vault […]

Rehabilitation aims to facilitate recovery from loss of function. Loss may be due to fracture, amputation, stroke or another neurologic disorder, arthritis, cardiac impairment, or prolonged deconditioning (eg, after some disorders and surgical procedures). Rehabilitation may involve physical, occupational, and speech therapy; psychologic counseling; and social services. For some patients, the goal is complete recovery […]

Exercise stimulates tissue change and adaptation (eg, increase in muscle mass and strength, cardiovascular endurance), whereas rest and recovery allow such change and adaptation to occur. Recovery from exercise is as important as the exercise stimulus. Regular physical activity reduces the likelihood of medical illness, decreases the incidence of the major causes of death, and […]

About 1 in 30 people traveling abroad requires emergency care. Illness in a foreign country may involve significant difficulties. Many insurance plans, including Medicare, are not valid in foreign countries; overseas hospitals often require a substantial cash deposit for nonresidents, regardless of insurance. Travel insurance plans, including some that arrange for emergency evacuation, are available […]

Air travel can cause or worsen certain medical problems; some are considered a contraindication to flight (see Table 1: Medical Aspects of Travel: Contraindications to Flying), and others may cause discomfort. Serious complications are rare. During a flight, any health care practitioner among the passengers may be asked to help fellow passengers who become ill. […]

At least 75% of people age > 65 yr do not exercise at recommended levels despite the known health benefits of exercise Longer survival Improved quality of life (eg, endurance, strength, mood, flexibility, cognitive function) Furthermore, many elderly people are not aware of how hard to exercise and also do not appreciate how much exercise […]

Suicidal behavior includes 3 types of self-destructive acts: completed suicide, attempted suicide, and suicide gestures. Thoughts and plans about suicide are referred to as suicide ideation. Completed suicide is a suicidal act that results in death. Attempted suicide is an act intended to be self-lethal, but one that does not result in death. Frequently, suicide […]

Generalized anxiety disorder (GAD) is characterized by excessive, almost daily anxiety and worry for ≥ 6 mo about many activities or events. The cause is unknown, although it commonly coexists in people who have alcohol abuse, major depression, or panic disorder. Diagnosis is based on history and physical examination. Treatment is psychotherapy, drug therapy, or […]

Everyone periodically experiences fear and anxiety. Fear is an emotional, physical, and behavioral response to an immediately recognizable external threat (eg, an intruder, a car spinning on ice). Anxiety is a distressing, unpleasant emotional state of nervousness and uneasiness; its causes are less clear. Anxiety is less tied to the exact timing of a threat; […]

_ _ _ Hyponatremia is decrease in serum Na concentration < 136 mEq/L caused by an excess of water relative to solute. Common causes include diuretic use, diarrhea, heart failure, and renal disease. Clinical manifestations are primarily neurologic (due to an osmotic shift of water into brain cells causing edema), especially in acute hyponatremia, and […]

Some drug categories (eg, analgesics, anticoagulants, antihypertensives, antiparkinsonian drugs, diuretics, hypoglycemic drugs, psychoactive drugs) pose special risks for elderly patients. Some, although reasonable for use in younger adults, are so risky as to be considered inappropriate for the elderly. The Beers Criteria are most commonly used to identify such inappropriate drugs (see Table 5: Drug […]

Drug-related problems include Adverse effects Ineffectiveness Adverse drug effects are effects that are unwanted, uncomfortable, or dangerous. Common examples are oversedation, confusion, hallucinations, falls, and bleeding. Among ambulatory people ≥ 65, adverse drug effects occur at a rate of about 50 events per 1000 person-years. Hospitalization rates due to adverse drug effects are 4 times […]

The most commonly reported sleep-related symptoms are insomnia and excessive daytime sleepiness (EDS). Insomnia is difficulty falling or staying asleep or a sensation of unrefreshing sleep. EDS is the tendency to fall asleep during normal waking hours. Insomnia and EDS are not disorders themselves but are symptoms of various sleep-related disorders. Parasomnias are abnormal sleep-related […]

Parkinson’s disease is an idiopathic, slowly progressive, degenerative CNS disorder characterized by resting tremor, muscular rigidity, slow and decreased movement, and postural instability. Diagnosis is clinical. Treatment is with levodopa plus carbidopa, other drugs, and, for refractory symptoms, surgery. Parkinson’s disease affects about 0.4% of people > 40 yr, 1% of people ≥ 65 yr, […]

Diagnostic procedures should not be used for preliminary screening, except perhaps in emergencies when a complete neurologic evaluation is impossible. Evidence uncovered during the history and physical examination should guide testing. Lumbar puncture (spinal tap): Lumbar puncture is used to evaluate intracranial pressure and CSF composition (see Table 1: Approach to the Neurologic Patient: Cerebrospinal […]

Patients with neurologic symptoms are approached in a stepwise manner termed the neurologic method, which consists of the following: Identifying the anatomic location of the lesion or lesions causing symptoms Identifying the pathophysiology involved Generating a differential diagnosis Selecting specific, appropriate tests Identifying the anatomy and pathophysiology of the lesion through careful history taking and […]

A muscle cramp (charley horse) is a sudden, brief, involuntary, painful contraction of a muscle or group of muscles. Cramps commonly occur in healthy people (usually middle-aged and elderly people), sometimes during rest, but particularly during or after exercise or at night (including during sleep—see Sleep and Wakefulness Disorders: Sleep-related leg cramps). Leg cramps at […]

“Numbness” can be used by patients to describe various symptoms, including loss of sensation, abnormal sensations, and weakness or paralysis. However, numbness is actually loss of sensation, either partial (hypesthesia) or complete (anesthesia). Numbness may involve the 3 major sensory modalities—light touch, pain and temperature sensation, and position and vibration sensation—to the same or different […]