In recognizing the signs and symptoms of neurological problems, it is first In some instances, you might experience emotional symptoms while in other cases . Structural, biochemical or electrical abnormalities in the brain, spinal cord or other nerves can result in a range of symptoms. Examples of symptoms include. Learn about neurologic diseases, including their symptoms, causes, and in languages other than English on Neurologic Diseases.
Neurological Symptoms Other
Arteriovenous Malformation - AVMs are defects of the circulatory system that are generally believed to arise during embryonic or fetal development or soon after birth. Although AVMs can develop in many different sites, those located in the brain or spinal cord can have especially widespread effects on the body. Most people with neurological AVMs experience few, if any, significant symptoms. The malformations tend to be discovered only incidentally, usually either at autopsy or during treatment for an unrelated disorder.
Asperger Syndrome - also called Asperger's syndrome, Asperger's disorder, Asperger's or AS is one of several autism spectrum disorders ASD characterized by difficulties in social interaction and by restricted, stereotyped interests and activities.
AS is distinguished from the other ASDs in having no general delay in language or cognitive development. Ataxia - Friedreich's ataxia is an inherited disease that causes progressive damage to the nervous system resulting in symptoms ranging from gait disturbance and speech problems to heart disease.
Coordination problems such as clumsy or awkward movements and unsteadiness, occurs in many different diseases and conditions. The ataxia of Friedreich's ataxia results from the degeneration of nerve tissue in the spinal cord and of nerves that control muscle movement in the arms and legs. The spinal cord becomes thinner and nerve cells lose some of their myelin sheath, the insular covering on all nerve cells that helps conduct nerve impulses.
Ataxia Telangiectasia - is a rare, childhood neurological disorder that causes degeneration in the part of the brain that controls motor movements and speech.
Its most unusual symptom is an acute sensitivity to ionizing radiation, such as X-rays or gamma-rays. The first signs of the disease, which include delayed development of motor skills, poor balance, and slurred speech, usually occur during the first decade of life.
Ataxias and Cerebellar or Spinocerebellar Degeneration - SCA is a genetic disease with multiple types, each of which could be considered a disease in its own right.
Spinocerebellar ataxia SCA is one of a group of genetic disorders characterized by slowly progressive in-coordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. Atrial Fibrillation and Stroke - Atrial fibrillation is a rapid uncoordinated generation of electrical impulses by the atria of the heart.
The most serious side effect of atrial fibrillation is stroke. Half of all strokes associated with atrial fibrillation are major and disabling.
Autism - is a brain development disorder that impairs social interaction and communication, and causes restricted and repetitive behavior, all starting before a child is three years old. This set of signs distinguishes autism from milder autism spectrum disorders ASD such as Asperger syndrome. Autism is highly heritable, although the genetics of autism are complex and it is generally unclear which genes are responsible.
Autonomic Dysfunction - refers to a disorder of autonomic nervous system ANS function. Most physicians view dysautonomia in terms of failure of the sympathetic or parasympathetic components of the ANS, but dysautonomia involving excessive ANS activities also can occur. Dysautonomia can be local, as in reflex sympathetic dystrophy, or generalized, as in pure autonomic failure.
It can be acute and reversible, as in Guillain-Barre syndrome, or chronic and progressive. Several common conditions such as diabetes and alcoholism can include dysautonomia. Back Pain - also known "dorsalgia" is pain felt in the back that may originate from the muscles, nerves, bones, joints or other structures in the spine. Barth Syndrome - is a rare congenital metabolic and neuromuscular disorder that affects boys.
It is passed from mother to son through the sex-linked, or X, chromosome. Symptoms affect multiple systems of the body and may include changes to metabolism, motor delays, hypotonia, delayed growth, cardiomyopathy, weakened immune system, chronic fatigue, lack of stamina, hypoglycemia, mouth ulcers, diarrhea, and varying degrees of physical and learning disability.
Batten Disease - is a rare, fatal, autosomal recessive neurodegenerative disorder that begins in childhood. Also known as Spielmeyer-Vogt-Sjogren-Batten disease, it is the most common form of a group of disorders called neuronal ceroid lipofuscinosis or NCLs. Becker's Myotonia - Myotonia congenita is an inherited neuromuscular disorder characterized by the inability of muscles to quickly relax after a voluntary contraction.
The condition is present since early childhood, but symptoms can be mild. Most children will be 2 or 3 years old when parents first notice their muscle stiffness, particularly in the legs, often provoked by sudden activity after rest. Behcet's Disease - is a chronic condition due to disturbances in the body's immune system. This system, which normally protects the body against infections through controlled inflammation, becomes over-active and produces unpredictable outbreaks of exaggerated inflammation.
This extra inflammation affects blood vessels, usually the small ones. As a result, symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a blood supply. Bell's Palsy - is a weakness or paralysis of the muscles that control expression on one side of your face. The disorder results from damage to one of a pair of facial nerves that runs beneath each ear to the muscles in your face.
Benign Essential Blepharospasm - BEB is a progressive neurological disorder characterized by involuntary muscle contractions and spasms of the eyelid muscles.
It is a form of dystonia, a movement disorder in which muscle contractions cause sustained eyelid closure, twitching or repetitive movements. Benign Focal Amyotrophy - Monomelic amyotrophy MMA is a rare disease of the nerves that control voluntary movements of the limbs. Monomelic amyotrophy may also be known as benign focal amyotrophy, single limb atrophy, Hirayama syndrome or Sobue disease. Descriptive terms such as brachial monomelic amyotrophy MMA confined to an arm or monomelic amyotrophy of the lower limb MMMA of a leg may be used to specify the type of limb affected.
Benign Intracranial Hypertension - sometimes called benign intracranial hypertension BIH or pseudotumor cerebri PTC is a neurological disorder that is characterized by increased intracranial pressure ICP , in the absence of a tumor or other diseases affecting the brain or its lining.
The main symptoms are headache and visual problems. Diagnosis requires brain scans and lumbar puncture. There are various medical and surgical treatments. Bernhardt-Roth Syndrome - is a disorder characterized by tingling, numbness, and burning pain in the outer side of the thigh.
The disorder is caused by compression of the lateral femoral cutaneous nerve as it exits the pelvis. It more commonly occurs in men than women, and is generally found in middle-aged or overweight individuals. People with the disorder frequently report that it appears or worsens after walking or standing.
The skin is often sensitive to touch. Meralgia paresthetica is associated with clothing that is too tight, pregnancy, diabetes, and obesity.
Binswanger's Disease - or Subcortical Leukoencephalopathy is a rare form of multi-infarct dementia caused by damage to deep white brain matter. It is characterized by loss of memory and intellectual function and by changes in mood. Blepharospasm - eye twitching is any abnormal tic or twitch of the eyelid.
However, it is normally distinguished from less serious disorders and refers to Benign Essential Blepharospasm, a focal dystonia a neurological movement disorder involving involuntary and sustained muscle contractions of the muscles around the eyes. The cause is often undetermined, but fatigue or an irritant are possible contributing factors. Bloch-Sulzberger Syndrome - Incontinentia pigmenti IP is one of a group of gene-linked diseases known as neurocutaneous disorders.
These disorders cause characteristic patterns of discolored skin and also involve the brain, eyes, nails, and hair. Males are more severely affected than females. Brachial Plexus Injuries - The brachial plexus is a network of nerves that conducts signals from the spine to the shoulder, arm, and hand.
Brachial plexus injuries are caused by damage to those nerves. Erb-Duchenne Erb's palsy refers to paralysis of the upper brachial plexus. Dejerine-Klumpke Klumpke's palsy refers to paralysis of the lower brachial plexus.
Symptoms of brachial plexus injury may include a limp or paralyzed arm; lack of muscle control in the arm, hand, or wrist, and lack of feeling or sensation in the arm or hand. Bradbury-Eggleston Syndrome - Orthostatic hypotension is a sudden fall in blood pressure that occurs when a person assumes a standing position. It may be caused by hypovolemia a decreased amount of blood in the body , resulting from the excessive use of diuretics, vasodilators, or other types of drugs, dehydration, or prolonged bed rest.
The disorder may be associated with Addison's disease, atherosclerosis build-up of fatty deposits in the arteries , diabetes, and certain neurological disorders.
Brain and Spinal Tumors - are abnormal growths of tissue found inside the skull or the bony spinal column, which are the primary components of the central nervous system CNS. Benign tumors are noncancerous, and malignant tumors are cancerous. The CNS is housed within rigid, bony quarters i. Tumors that originate in the brain or spinal cord are called primary tumors.
Brain Aneurysm - A cerebral aneurysm or brain aneurysm is a cerebrovascular disorder in which weakness in the wall of a cerebral artery or vein causes a localized dilation or ballooning of the blood vessel. Brain Injury - TBI , traumatic injuries to the brain, also called intracranial injury, or simply head injury, occurs when a sudden trauma causes brain damage. TBI can result from a closed head injury or a penetrating head injury and is one of two subsets of acquired brain injury ABI.
The other subset is non-traumatic brain injury e. Parts of the brain that can be damaged include the cerebral hemispheres, cerebellum, and brain stem. TBI can cause a host of physical, cognitive, emotional, and social effects. Brown-Sequard Syndrome - is an incomplete spinal cord lesion characterized by a clinical picture reflecting hemisection of the spinal cord, often in the cervical cord region. It is a rare syndrome, consisting of ipsilateral hemiplegia with contralateral pain and temperature sensation deficits because of the crossing of the fibers of the spinothalamic tract.
Bulbospinal Muscular Atrophy - or Kennedy's disease is an inherited motor neuron disease that affects males. It is one of a group of disorders called spinal muscular atrophy SMA. Onset of the disease is usually between the ages of 20 and 40, although it has been diagnosed in men from their teens to their 70s.
Early symptoms include tremor of the outstretched hands, muscle cramps with exertion, and fasciculations fleeting muscle twitches visible under the skin. Cadasil - Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy is an inherited form of cerebrovascular disease that occurs when the thickening of blood vessel walls blocks the flow of blood to the brain.
The disease primarily affects small blood vessels in the white matter of the brain. A mutation in the Notch3 gene alters the muscular walls in these small arteries.
Other symptoms include white matter lesions throughout the brain, cognitive deterioration, seizures, vision problems, and psychiatric problems such as severe depression and changes in behavior and personality.
Individuals may also be at higher risk of heart attack. Canavan Disease - one of the most common cerebral degenerative diseases of infancy, is a gene-linked, neurological birth disorder in which the white matter of the brain degenerates into spongy tissue riddled with microscopic fluid-filled spaces.
Canavan disease is one of a group of genetic disorders known as the leukodystrophies. These diseases cause imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fibers in the brain. Myelin, which lends its color to the "white matter" of the brain, is a complex substance made up of at least ten different chemicals. Each of the leukodystrophies affects one and only one of these substances.
Canavan disease is caused by mutations in the gene for an enzyme called aspartoacylase. Symptoms of Canavan disease, which appear in early infancy and progress rapidly, may include mental retardation, loss of previously acquired motor skills, feeding difficulties, abnormal muscle tone floppiness or stiffness , and an abnormally large, poorly controlled head.
Carpal Tunnel Syndrome - occurs when the median nerve, which runs from the forearm into the hand, becomes pressed or squeezed at the wrist.
The median nerve controls sensations to the palm side of the thumb and fingers although not the little finger , as well as impulses to some small muscles in the hand that allow the fingers and thumb to move. The carpal tunnel - a narrow, rigid passageway of ligament and bones at the base of the hand houses the median nerve and tendons. Sometimes, thickening from irritated tendons or other swelling narrows the tunnel and causes the median nerve to be compressed.
The result may be pain, weakness, or numbness in the hand and wrist, radiating up the arm. The key symptom of CRPS is continuous, intense pain out of proportion to the severity of the injury, which gets worse rather than better over time. CRPS most often affects one of the arms, legs, hands, or feet. Often the pain spreads to include the entire arm or leg.
Typical features include dramatic changes in the color and temperature of the skin over the affected limb or body part, accompanied by intense burning pain, skin sensitivity, sweating, and swelling. Cavernomas - A cerebral cavernous malformation CCM is a collection of small blood vessels capillaries in the central nervous system CNS that is enlarged and irregular in structure and takes the shape of a characteristic honeycomb-like pattern.
In CCM, the walls of the capillaries are thinner than normal, less elastic, and prone to leaking. Cavernous malformations can occur anywhere in the body, but usually only produce symptoms when they are found in the brain and spinal cord. Some people with CCM - experts estimate 25 percent - will never experience any related medical problems. Cavernous Angioma - also known as cerebral cavernous malformation CCM , cavernous haemangioma, and cavernoma, is a vascular disorder of the central nervous system that may appear either sporadically or exhibit autosomal dominant inheritance.
Central Cord Syndrome - is a form of incomplete spinal cord injury characterized by impairment in the arms and hands and to a lesser extent in the legs.
The brain's ability to send and receive signals to and from parts of the body below the site of injury is reduced but not entirely blocked. This syndrome is associated with damage to the large nerve fibers that carry information directly from the cerebral cortex to the spinal cord. These nerves are particularly important for hand and arm function.
Symptoms may include paralysis or loss of fine control of movements in the arms and hands, with relatively less impairment of leg movements. Sensory loss below the site of the injury and loss of bladder control may also occur. Central Pain Syndrome - is a neurological condition caused by damage to or dysfunction of the central nervous system CNS , which includes the brain, brainstem, and spinal cord. This syndrome can be caused by stroke, multiple sclerosis, tumors, epilepsy, brain or spinal cord trauma, or Parkinson's disease.
The character of the pain associated with this syndrome differs widely among individuals partly because of the variety of potential causes. Central pain syndrome may affect a large portion of the body or may be more restricted to specific areas, such as hands or feet. The extent of pain is usually related to the cause of the CNS injury or damage. Pain is typically constant, may be moderate to severe in intensity, and is often made worse by touch, movement, emotions, and temperature changes, usually cold temperatures.
Central Pontine Myelinolysis - CPM is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells.
Through a mechanism that is only partly understood, the shift in water and brain molecules leads to the destruction of myelin, a substance that surrounds and protects nerve fibers. Nerve cells neurons can also be damaged.
Certain areas of the brain are particularly susceptible to myelinolysis, especially the part of the brainstem called the pons. Some individuals will also have damage in other areas of the brain, which is called extrapontine myelinolysis EPM. Cephalic Disorders - are congenital conditions that stem from damage to, or abnormal development of, the budding nervous system. Cephalic is a term that means "head" or "head end of the body.
Although there are many congenital developmental disorders, this fact sheet briefly describes only cephalic conditions. Cephalic disorders are not necessarily caused by a single factor but may be influenced by hereditary or genetic conditions or by environmental exposures during pregnancy such as medication taken by the mother, maternal infection, or exposure to radiation.
Some cephalic disorders occur when the cranial sutures the fibrous joints that connect the bones of the skull join prematurely. Most cephalic disorders are caused by a disturbance that occurs very early in the development of the fetal nervous system. Ceramidase Deficiency - Farber's disease describes a group of inherited metabolic disorders called lipid storage diseases, in which excess amounts of lipids oils, fatty acids, and related compounds build up to harmful levels in the joints, tissues, and central nervous system.
The liver, heart, and kidneys may also be affected. Symptoms are typically seen in the first few weeks of life and include impaired motor and mental ability and difficulty with swallowing.
Other symptoms may include arthritis, swollen lymph nodes and joints, hoarseness, nodules under the skin and sometimes in the lungs and other parts of the body , chronic shortening of muscles or tendons around joints, and vomiting. Affected persons may require the insertion of a breathing tube. In severe cases, the liver and spleen are enlarged. Cerebellar Degeneration - is a disease process in which neurons in the cerebellum - the area of the brain that controls muscle coordination and balance - deteriorate and die.
Diseases that cause cerebellar degeneration can also involve areas of the brain that connect the cerebellum to the spinal cord, such as the medulla oblongata, the cerebral cortex, and the brain stem. Cerebellar degeneration is most often the result of inherited genetic mutations that alter the normal production of specific proteins that are necessary for the survival of neurons. Cerebellar Hypoplasia - is a neurological condition in which the cerebellum is smaller than usual or not completely developed.
Cerebellar hypoplasia is a feature of a number of congenital present at birth malformation syndromes, such as Walker-Warburg syndrome. It is also associated with several inherited metabolic disorders, such as Williams syndrome, and some of the neurodegenerative disorders that begin in early childhood, such as ataxia telangiectasia. In an infant or young child, symptoms of a disorder that features cerebellar hypoplasia might include floppy muscle tone, developmental or speech delay, problems with walking and balance, seizures, mental retardation, and involuntary side to side movements of the eyes.
In an older child, symptoms might include headache, dizzy spells, clumsiness, and hearing impairment. Cerebral Aneurysm - also known as an intracranial or intracerebral aneurysm is a weak or thin spot on a blood vessel in the brain that balloons out and fills with blood. The bulging aneurysm can put pressure on a nerve or surrounding brain tissue. It may also leak or rupture, spilling blood into the surrounding tissue called a hemorrhage.
Some cerebral aneurysms, particularly those that are very small, do not bleed or cause other problems. Cerebral aneurysms can occur anywhere in the brain, but most are located along a loop of arteries that run between the underside of the brain and the base of the skull.
Cerebral Arteriosclerosis - Cerebral arteriosclerosis is the result of thickening and hardening of the walls of the arteries in the brain. Symptoms of cerebral arteriosclerosis include headache, facial pain, and impaired vision. Cerebral arteriosclerosis can cause serious health problems. If the walls of an artery are too thick, or a blood clot becomes caught in the narrow passage, blood flow to the brain can become blocked and cause an ischemic stroke.
When the thickening and hardening is uneven, arterial walls can develop bulges called aneurysms. If a bulge ruptures, bleeding in the brain can cause a hemorrhagic stroke. Both types of stroke can be fatal. Cerebral Atrophy - is a common feature of many of the diseases that affect the brain. Atrophy of any tissue means loss of cells. In brain tissue, atrophy describes a loss of neurons and the connections between them. Atrophy can be generalized, which means that all of the brain has shrunk; or it can be focal, affecting only a limited area of the brain and resulting in a decrease of the functions that area of the brain controls.
If the cerebral hemispheres the two lobes of the brain that form the cerebrum are affected, conscious thought and voluntary processes may be impaired. Cerebral Beriberi - Wernicke's encephalopathy is a degenerative brain disorder caused by the lack of thiamine vitamin B1. It may result from alcohol abuse, dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy.
Symptoms include mental confusion, vision impairment, stupor, coma, hypothermia, hypotension, and ataxia.
Korsakoff's amnesic syndrome-a memory disorder-also results from a deficiency of thiamine, and is associated with alcoholism.
The heart, vascular, and nervous system are involved. Symptoms include amnesia, confabulation, attention deficit, disorientation, and vision impairment. The main features of Korsakoff's amnesic syndrome are the impairments in acquiring new information or establishing new memories, and in retrieving previous memories.
Cerebral Gigantism - Sotos syndrome is a rare genetic disorder characterized by excessive physical growth during the first 2 to 3 years of life. The disorder may be accompanied by mild mental retardation, delayed motor, cognitive, and social development, hypotonia low muscle tone , and speech impairments. Children with Sotos syndrome tend to be large at birth and are often taller, heavier, and have larger heads macrocrania than is normal for their age.
Symptoms of the disorder, which vary among individuals, include a disproportionately large and long head with a slightly protrusive forehead, large hands and feet, hypertelorism an abnormally increased distance between the eyes , and down-slanting eyes.
Clumsiness, an awkward gait, and unusual aggressiveness or irritability may also occur. Cerebral Hypoxia - refers to a condition in which there is a decrease of oxygen supply to the brain even though there is adequate blood flow. Drowning, strangling, choking, suffocation, cardiac arrest, head trauma, carbon monoxide poisoning, and complications of general anesthesia can create conditions that can lead to cerebral hypoxia. Symptoms of mild cerebral hypoxia include inattentiveness, poor judgment, memory loss, and a decrease in motor coordination.
Brain cells are extremely sensitive to oxygen deprivation and can begin to die within five minutes after oxygen supply has been cut off. When hypoxia lasts for longer periods of time, it can cause coma, seizures, and even brain death.
In brain death, there is no measurable activity in the brain, although cardiovascular function is preserved. Life support is required for respiration. Cerebral Palsy - The term cerebral palsy refers to any one of a number of neurological disorders that appear in infancy or early childhood and permanently affect body movement and muscle coordination but don't worsen over time.
Even though cerebral palsy affects muscle movement, it isn't caused by problems in the muscles or nerves. It is caused by abnormalities in parts of the brain that control muscle movements. The majority of children with cerebral palsy are born with it, although it may not be detected until months or years later. The early signs of cerebral palsy usually appear before a child reaches 3 years of age. The most common are a lack of muscle coordination when performing voluntary movements ataxia ; stiff or tight muscles and exaggerated reflexes spasticity ; walking with one foot or leg dragging; walking on the toes, a crouched gait, or a "scissored" gait; and muscle tone that is either too stiff or too floppy.
It is characterized by craniofacial and skeletal abnormalities, severely reduced muscle tone, and impairment of reflexes. Symptoms may include large, low-set ears, small eyes, microcephaly abnormal smallness of the head , micrognathia abnormal smallness of the jaws , clenched fists, wide-set nipples, vision impairments, involuntary eye movements, and mental retardation, which can be moderate or severe.
Respiratory infections are frequent. COFS is diagnosed at birth. Charcot-Marie-Tooth Disease - CMT is one of the most common inherited neurological disorders, affecting approximately 1 in 2, people in the United States. CMT, also known as hereditary motor and sensory neuropathy HMSN or peroneal muscular atrophy, comprises a group of disorders that affect peripheral nerves.
The peripheral nerves lie outside the brain and spinal cord and supply the muscles and sensory organs in the limbs. Disorders that affect the peripheral nerves are called peripheral neuropathies. Chiari Malformation - CMs are structural defects in the cerebellum, the part of the brain that controls balance. When the indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid the liquid that surrounds and protects the brain and spinal cord and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination.
There are three primary types of CM. The most common is Type I, which may not cause symptoms and is often found by accident during an examination for another condition. Type II also called Arnold-Chiari malformation is usually accompanied by a myelomeningocele-a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back.
This can cause partial or complete paralysis below the spinal opening. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia, and spinal curvature. Chorea - is an abnormal voluntary movement disorder, one of a group of neurological disorders called dyskinesias, which are caused by over-activity of the neurotransmitter dopamine in the areas of the brain that control movement. Chorea is characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.
Chorea often occurs with athetosis, which adds twisting and writhing movements. Chorea is a primary feature of Huntington's disease, a progressive, hereditary movement disorder that appears in adults, but it may also occur in a variety of other conditions.
Syndenham's chorea occurs in a small percentage 20 percent of children and adolescents as a complication of rheumatic fever. Chronic Inflammatory Demyelinating Polyneuropathy CIDP - is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms.
The disorder, which is sometimes called chronic relapsing polyneuropathy, is caused by damage to the myelin sheath the fatty covering that wraps around and protects nerve fibers of the peripheral nerves. Although it can occur at any age and in both genders, CIDP is more common in young adults, and in men more so than women.
It often presents with symptoms that include tingling or numbness beginning in the toes and fingers , weakness of the arms and legs, loss of deep tendon reflexes areflexia , fatigue, and abnormal sensations.
CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic counterpart of that acute disease. Chronic Pain - While acute pain is a normal sensation triggered in the nervous system to alert you to possible injury and the need to take care of yourself, chronic pain is different.
Pain signals keep firing in the nervous system for weeks, months, even years. There may have been an initial mishap - sprained back, serious infection, or there may be an ongoing cause of pain - arthritis, cancer, ear infection, but some people suffer chronic pain in the absence of any past injury or evidence of body damage.
Many chronic pain conditions affect older adults. Common chronic pain complaints include headache, low back pain, cancer pain, arthritis pain, neurogenic pain pain resulting from damage to the peripheral nerves or to the central nervous system itself , psychogenic pain pain not due to past disease or injury or any visible sign of damage inside or outside the nervous system.
Coffin Lowry Syndrome - is a rare genetic disorder characterized by craniofacial head and facial and skeletal abnormalities, mental retardation, short stature, and hypotonia. Characteristic facial features may include an underdeveloped upper jaw bone maxillary hypoplasia , a broad nose, protruding nostrils nares , an abnormally prominent brow, down-slanting eyelid folds palpebral fissures , widely spaced eyes hypertelorism , large ears, and unusually thick eyebrows.
Skeletal abnormalities may include abnormal front-to-back and side-to-side curvature of the spine kyphoscoliosis , unusual prominence of the breastbone pectus carinatum , and short, hyper-extensible, tapered fingers. Additional abnormalities may also be present. Other features may include feeding and respiratory problems, developmental delay, mental retardation, hearing impairment, awkward gait, flat feet, and heart and kidney involvement. Colpocephaly - is a congenital brain abnormality in which the occipital horns - the posterior or rear portion of the lateral ventricles cavities of the brain - are larger than normal because white matter in the posterior cerebrum has failed to develop or thicken.
Colpocephaly, one of a group of structural brain disorders known as cephalic disorders, is characterized by microcephaly an abnormally small head and mental retardation. Other features may include movement abnormalities, muscle spasms, and seizures. Although the cause of colpocephaly is unknown, researchers believe that the disorder results from some kind of disturbance in the fetal environment that occurs between the second and sixth months of pregnancy.
Colpocephaly may be diagnosed late in pregnancy, although it is often misdiagnosed as hydrocephalus excessive accumulation of cerebrospinal fluid in the brain. Coma and Persistent Vegetative State - A coma is a profound or deep state of unconsciousness. An individual in a state of coma is alive but unable to move or respond to his or her environment. Coma may occur as a complication of an underlying illness, or as a result of injuries, such as head trauma. A persistent vegetative state commonly, but incorrectly, referred to as "brain-death" sometimes follows a coma.
Individuals in such a state have lost their thinking abilities and awareness of their surroundings, but retain non-cognitive function and normal sleep patterns. Even though those in a persistent vegetative state lose their higher brain functions, other key functions such as breathing and circulation remain relatively intact.
Spontaneous movements may occur, and the eyes may open in response to external stimuli. Congenital Facial Diplegia - Mobius syndrome is a rare birth defect caused by the absence or underdevelopment of the 6th and 7th cranial nerves, which control eye movements and facial expression.
The first symptom, present at birth, is an inability to suck. Other symptoms can include: Small or absent brain stem nuclei that control the cranial nerves, as well as decreased numbers of muscle fibers, have been reported.
Deformities of the tongue, jaw, and limbs, such as clubfoot and missing or webbed fingers, may also occur. As children get older, lack of facial expression and inability to smile become the dominant visible symptoms. The prognosis for otherwise normal development is excellent in most cases.
Congenital Myasthenia - is an inherited disorder that affects the transmission of signals to the muscles. It results from a variety of genetic defects at the molecules associated with neuromuscular transmission. Congenital myasthenia is not the same as myasthenia gravis, which is an autoimmune disorder.
More than a dozen congenital myasthenic syndromes have been classified. Symptoms are usually noticed in early childhood and include drooping eyelids, facial weakness, and limb weakness. Parents of children with congenital myasthenia frequently show no symptoms of the disorder. Congenital Myopathy - is a term for any muscle disorder present at birth.
By this definition the congenital myopathies could include hundreds of distinct neuromuscular syndromes and disorders. In general, congenital myopathies cause loss of muscle tone and muscle weakness in infancy and delayed motor milestones, such as walking, later in childhood. Three distinct disorders are definitively classified as congenital myopathies: Central core disease is a dominantly inherited genetic disease characterized by mild leg weakness appearing in infancy.
This weakness does not progress with age, but leads to delay in walking. Nemaline rod myopathy is a dominantly or recessively inherited, genetic disease characterized by infantile muscle weakness and loss of muscle tone, accompanied by problems in suckling or feeding, delay in walking, and occasionally respiratory problems.
Corticobasal Degeneration - is a progressive neurological disorder characterized by nerve cell loss and atrophy shrinkage of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually.
Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body unilateral , but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia an absence of movements , rigidity a resistance to imposed movement , disequilibrium impaired balance ; and limb dystonia abnormal muscle postures.
Other symptoms such as cognitive and visual-spatial impairments, apraxia loss of the ability to make familiar, purposeful movements , hesitant and halting speech, myoclonus muscular jerks , and dysphagia difficulty swallowing may also occur.
An individual with corticobasal degeneration eventually becomes unable to walk. Cranial Arteritis - Vasculitis is an inflammation of the vascular system, which includes the veins, arteries, and capillaries. Dysfunction may occur due to the inflammation itself or over time as the blood vessel walls swell, harden, thicken, and develop scar tissue.
This narrows the passage through which blood can flow. As the condition progresses, it can slow or completely stop the normal flow of blood. A vasculitis syndrome may begin suddenly or develop over time.
Symptoms include headaches, fever, malaise feeling out-of-sorts , rapid weight loss, confusion or forgetfulness, aches and pains in the joints and muscles, pain while chewing or swallowing, paralysis or numbness in the arms or legs, double vision, blurred vision, or blindness.
Craniosynostosis - is a birth defect of the brain characterized by the premature closure of one or more of the fibrous joints between the bones of the skull called the cranial sutures before brain growth is complete.
Closure of a single suture is most common. The abnormally shaped skull that results is due to the brain not being able to grow in its natural shape because of the closure. Instead it compensates with growth in areas of the skull where the cranial sutures have not yet closed. The condition can be gene-linked, or caused by metabolic diseases, such as rickets or an over-active thyroid. Some cases are associated with other disorders such as microcephaly abnormally small head and hydrocephalus excessive accumulation of cerebrospinal fluid in the brain.
The first sign of craniosynostosis is an abnormally shaped skull. Other features can include signs of increased intracranial pressure, developmental delays, or mental retardation, which are caused by constriction of the growing brain.
Seizures and blindness may also occur. It affects about one person in every one million people per year worldwide; in the United States there are about cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within 1 year.
In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. Cumulative Trauma Disorders - Repetitive motion disorders RMDs are a family of muscular conditions that result from repeated motions performed in the course of normal work or daily activities. RMDs include carpal tunnel syndrome, bursitis, tendonitis, epicondylitis, ganglion cyst, tenosynovitis, and trigger finger.
RMDs are caused by too many uninterrupted repetitions of an activity or motion, unnatural or awkward motions such as twisting the arm or wrist, overexertion, incorrect posture, or muscle fatigue. RMDs occur most commonly in the hands, wrists, elbows, and shoulders, but can also happen in the neck, back, hips, knees, feet, legs, and ankles.
The disorders are characterized by pain, tingling, numbness, visible swelling or redness of the affected area, and the loss of flexibility and strength. For some individuals, there may be no visible sign of injury, although they may find it hard to perform easy tasks Over time, RMDs can cause temporary or permanent damage to the soft tissues in the body - such as the muscles, nerves, tendons, and ligaments - and compression of nerves or tissue. Cushing's Syndrome - also called hypercortisolism, is a rare endocrine disorder caused by chronic exposure of the body's tissues to excess levels of cortisol - a hormone naturally produced by the adrenal gland.
Exposure to too much cortisol can occur from long-term use of synthetic glucocorticoid hormones to treat inflammatory illnesses. Pituitary adenomas benign tumors of the pituitary gland that secrete increased amounts of ACTH adrenocorticotropic hormone, a substance that controls the release of cortisol can also spur overproduction of cortisol. Tumors of the adrenal gland and ectopic ACTH syndrome a condition in which ACTH is produced by various types of potentially malignant tumors that occur in different parts of the body can cause similar problems with cortisol balance.
Common symptoms of Cushing's syndrome include upper body obesity, severe fatigue and muscle weakness, high blood pressure, backache, elevated blood sugar, easy bruising, and bluish-red stretch marks on the skin. In women, there may be increased growth of facial and body hair, and menstrual periods may become irregular or stop completely. Neurological symptoms include difficulties with memory and neuromuscular disorders.
Cytomegalic Inclusion Body Disease - The cytomegalovirus CMV is a virus found universally throughout the world that infects between 50 to 80 percent of all adults in the United States by the age of CMV is in the same family of viruses that includes herpes simplex types 1 and 2, and the viruses that cause infectious mononucleosis EBV , chickenpox, and shingles.
A hallmark of CMV is the reappearance of symptoms throughout life, as the virus cycles through periods of dormancy and active infection. Most people who acquire the virus as children or adults display no signs or have mild symptoms and no long-term health consequences. Those who do have symptoms experience mononucleosis-like indications, such as a prolonged fever, fatigue, mild hepatitis, and tender lymph nodes.
Dancing Eyes-Dancing Feet Syndrome - Opsoclonus myoclonus is a rare neurological disorder characterized by an unsteady, trembling gait, myoclonus brief, shock-like muscle spasms , and opsoclonus irregular, rapid eye movements. Other symptoms may include difficulty speaking, poorly articulated speech, or an inability to speak.
A decrease in muscle tone, lethargy, irritability, and malaise a vague feeling of bodily discomfort may also be present. Dandy-Walker Syndrome - DWS , or Dandy-Walker complex, is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it.
The Dandy-Walker complex is a genetically sporadic disorder that occurs one in every 25, live births, mostly in females. Dawson Disease - Subacute sclerosing panencephalitis SSPE is a chronic persistent infection of the central nervous system caused by an altered form of the measles virus.
It can occur anywhere from 2 to 10 years after the original measles illness, and generally results in progressive neurological deterioration due to brain inflammation and nerve cell death. Since the widespread use of the measles vaccine, SSPE has become very rare. Dementia - is the progressive decline in cognitive function due to damage or disease in the brain beyond what might be expected from normal aging.
Although dementia is far more common in the geriatric population, it may occur in any stage of adulthood. MID is caused by multiple strokes disruption of blood flow to the brain.
Disruption of blood flow leads to damaged brain tissue. Some of these strokes may occur without noticeable clinical symptoms. Doctors refer to these as "silent strokes.
Symptoms include confusion or problems with short-term memory; wandering, or getting lost in familiar places; walking with rapid, shuffling steps; losing bladder or bowel control; laughing or crying inappropriately.
Dementia - Semantic - Frontotemporal dementia FTD describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick's disease, the name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick's disease, primary progressive aphasia, and semantic dementia as FTD.
Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex. Dementia - Subcortical - Binswanger's disease BD , also called subcortical vascular dementia, is a type of dementia caused by widespread, microscopic areas of damage to the deep layers of white matter in the brain. The damage is the result of the thickening and narrowing atherosclerosis of arteries that feed the subcortical areas of the brain.
Atherosclerosis commonly known as "hardening of the arteries" is a systemic process that affects blood vessels throughout the body. It begins late in the fourth decade of life and increases in severity with age. As the arteries become more and more narrowed, the blood supplied by those arteries decreases and brain tissue dies. The central feature of DLB is progressive cognitive decline, combined with three additional defining features: Dentate Cerebellar Ataxia - Dyssynergia Cerebellaris Myoclonica refers to a collection of rare, degenerative, neurological disorders characterized by epilepsy, cognitive impairment, myoclonus, and progressive ataxia.
Symptoms include seizures, tremor, and reduced muscle coordination. Onset of the disorder generally occurs in early adulthood. Tremor may begin in one extremity and later spread to involve the entire voluntary muscular system. Arms are usually more affected than legs. Some of the cases are due to mitochondrial abnormalities. Dentatorubral Atrophy - Dentatorubral pallidoluysian atrophy is a rare neurodegenerative disorder characterized by cerebellar ataxia, myoclonic epilepsy, choreoathetosis, and dementia.
The disorder is related to the expansion of a trinucleotide repeat within this gene. The encoded protein includes a serine repeat and a region of alternating acidic and basic amino acids, as well as the variable glutamine repeat. Alternative splicing results in two transcripts variants that encode the same protein. Dermatomyositis - is connective-tissue disease that is characterized by inflammation of the muscles and the skin. Its cause is unknown, but it may result from either a viral infection or an autoimmune reaction.
Developmental Dyspraxia - is one or all of a heterogeneous range of psychological development disorders affecting the initiation, organization and performance of action. It entails the partial loss of the ability to coordinate and perform certain purposeful movements and gestures in the absence of motor or sensory impairments. Devic's Syndrome - Neuromyelitis optica NMO is an uncommon disease syndrome of the central nervous system CNS that affects the optic nerves and spinal cord.
Individuals with NMO develop optic neuritis, which causes pain in the eye and vision loss, and transverse myelitis, which causes weakness, numbness, and sometimes paralysis of the arms and legs, along with sensory disturbances and loss of bladder and bowel control. NMO leads to loss of myelin, which is a fatty substance that surrounds nerve fibers and helps nerve signals move from cell to cell.
The syndrome can also damage nerve fibers and leave areas of broken-down tissue. In the disease process of NMO, for reasons that aren't yet clear, immune system cells and antibodies attack and destroy myelin cells in the optic nerves and the spinal cord.
Diabetic Neuropathy - is a peripheral nerve disorder caused by diabetes. The symptoms of diabetic neuropathy are often slight at first. In fact, some mild cases may go unnoticed for a long time. Numbness, pain, or tingling in the feet, or legs may, after several years, lead to weakness in the muscles of the feet. Occasionally, diabetic neuropathy can flare up suddenly and affect specific nerves so that an affected individual will develop double vision or drooping eyelids, or weakness and atrophy of the thigh muscles.
Nerve damage caused by diabetes generally occurs over a period of years and may lead to problems with the digestive tract and sexual organs, which can cause indigestion, diarrhea or constipation, dizziness, bladder infections, and impotence. Diffuse Sclerosis - Schilder's disease is not the same as Addison-Schilder disease adrenoleukodystrophy. Schilder's disease is a rare progressive demyelinating disorder which usually begins in childhood.
Symptoms may include dementia, aphasia, seizures, personality changes, poor attention, tremors, balance instability, incontinence, muscle weakness, headache, vomiting, and vision and speech impairment. The disorder is a variant of multiple sclerosis. Dravet Syndrome - also called severe myoclonic epilepsy of infancy SMEI , is a severe form of epilepsy. It appears during the first year of life with frequent febrile seizures - fever-related seizures that, by definition, are rare beyond age 5.
Later, other types of seizures typically arise, including myoclonus involuntary muscle spasms. Status epilepticus - a state of continuous seizure requiring emergency medical care - also may occur. Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others.
Dysautonomia - refers to a disorder of autonomic nervous system ANS function. Dysautonomia also can occur as a primary condition or in association with degenerative neurological diseases such as Parkinson's disease. Dysgraphia - is a neurological disorder characterized by writing disabilities. Specifically, the disorder causes a person's writing to be distorted or incorrect. In children, the disorder generally emerges when they are first introduced to writing.
They make inappropriately sized and spaced letters, or write wrong or misspelled words, despite thorough instruction. Children with the disorder may have other learning disabilities, however, they usually have no social or other academic problems. Cases of dysgraphia in adults generally occur after some trauma. In addition to poor handwriting, dysgraphia is characterized by wrong or odd spelling, and production of words that are not correct i.
The cause of the disorder is unknown. Dyslexia - is a specific learning disability that manifests primarily as a difficulty with written language, particularly with reading and spelling.
It is separate and distinct from reading difficulties resulting from other causes, such as a non-neurological deficiency with vision or hearing, or from poor or inadequate reading instruction. Recommendations on how to best evaluate and treat such patients have been drawn up by nursing groups Herr et al. However, none of these approaches include objective measures of pain, and rates of misdiagnosis of patients in the minimally conscious state motor cortex stimulation; some evidence of awareness of self and environment or vegetative state wakefulness without awareness Fins et al.
Attempts have been made to provide objective measures of cognition and pain using neuroimaging. PET studies show activation in regions of the pain network thalamus, S1 and the secondary somatosensory or insular, frontoparietal, and anterior cingulate cortices in patients with motor cortex stimulation and vegetative state at a lower level and demonstrate loss of functional connectivity between S1 and frontoparietal regions in both conditions Boly et al.
In an earlier study, pain stimulation activated the midbrain in the same regions activated by somatosensory stimulation , contralateral thalamus, and primary somatosensory cortex in all vegetative state patients, even in the absence of cortical evoked potentials Laureys et al.
The activation in the primary cortex seems to be isolated and dissociated from higher order associative cortices in vegetative state. As noted in the reports above, in comatose patients vegetative or minimally conscious state , pain stimuli activate well-defined pain pathways in the brain.
What is unclear is if the patient comprehends or suffers from pain. Behavioural scales have been used to try to evaluate pain in comatose patients, including the Nociceptive Coma Scale Schnakers et al. While neuroimaging studies may provide data relating to brain regions involved in aversion and affective dimensions of pain, currently we can utilize functional MRI methods to define nociceptive processing but not pain itself.
These data indicate that a better understanding of pain perception in the minimally conscious or vegetative state is a clinical and ethical imperative. Further studies are required, as well as the development of new neuroimaging modalities that can be applied easily at the bedside. Examples of these atypical presentations include non-dermatomal sensory deficit in patients who have pain Mailis-Gagnon et al. In addition, the evolution of pain in depressed patients with no prior history of pain or non-traumatic post-traumatic stress disorder provides further insight into the complexity of alterations in brain circuits producing pain.
Some patients display a recognizable illness without any pathology, making it difficult to determine if the illness is real or simulated.
Similarly puzzling are instances of delayed onset of pain following an insult and spontaneous resolution of pain, which may take place over a short time period even if pain has been present for years Schott, Finally, the effect of opioids in chronic pain is often counter-intuitive.
For example, they have relatively limited efficacy a decrease of 13 points on a point scale Eisenberg et al. For example, opioids induce migraine chronification Bigal and Lipton, Opioids clearly alter brain systems Upadhyay et al. Objective measures of pain would help clarify many of these issues and aid in the assessment of clinical efficacy of analgesics.
Despite trials of a number of approaches, chronic pain is largely refractory to treatment. Chronic pain associated with neurological disease has varied presentations and temporal profiles Fig. Below, I discuss therapeutic approaches that may be more logical in light of the new understanding that chronic pain is a disease of the brain.
The over-riding goal of these approaches is to restore brain networks to states that are adaptive. This discussion is not meant to suggest specific therapies for pain in particular neurological diseases, but rather to spur further exploration of new chronic pain treatment options that represent a fairly radical departure from our previous therapeutic approach.
Schematic of altered pain processing in neurological disease. The figure summarizes altered pain processing in examples of neurological disease, as well as underlying mechanisms that contribute to chronic disease-related pain. In most diseases, multiple regions are affected as opposed to secondary effects such as centralization of pain following a peripheral nerve injury. Whether or not the disease is a primary or secondary cause of pain, pain itself drives an altered brain state Fig.
Damage anywhere along the pain pathway from peripheral nerve to spinothalamic tracts and more central pathways including thalamus and thalamocortical projections may result in neuropathic pain. In the figure, damage as indicated by red crosses or abnormal activation in pain pathways circles , contributes to other changes in brain systems. Motor and sensory systems are well integrated Flor and Diers, It has been suggested that phantom limb pain is caused by motor cortex dysfunction that is the result of dissociation between motor and sensory representations Karl et al.
Through motor training, phantom-limb patients may decrease their pain, presumably by resetting these altered sensory—motor dissociations. Recent work has indicated that mirror movement differentially activates the sensory cortex in amputees with and without phantom pain, further implicating altered functional connectivity Diers et al.
Related to this is the use of smart limb prosthetics Marasco et al. Electroconvulsive therapy has been suggested to be of use in chronic neurological diseases such as Parkinson's disease—on both psychiatric manifestations and motor systems Popeo and Kellner, Electroconvulsive therapy has also been recommended for chronic pain Fukui et al. Thalamic blood flow is reportedly normalized following electroconvulsive therapy in patients with CRPS Fukui et al.
The interesting application in many of the neurological orders discussed relates to electroconvulsive therapy's strong antidepressant effects Merkl et al. Ketamine has been used to treat chronic pain and depression Berman et al. In chronic pain, higher doses seem to be more effective and reports have suggested that very high or continued dosing anaesthetic levels may reverse conditions such as CRPS Kiefer et al.
Indeed in uncontrolled trials, anaesthetic levels of ketamine reversed pain in 20 patients with chronic pain, producing complete relief in all patients at 1 month; pain relief persisted in 17 of these patients at 3 months, and in 16 at 6 months Kiefer et al.
Clearly, controlled trials are required to verify these findings, but they raise the exciting possibility that ketamine alters brain systems in a significant manner in a highly resistant population. This approach is clearly not recommended in patients with neurodegenerative disease, particularly those with hyperexcitability-related pathogenesis, in which ketamine could potentially exacerbate ongoing neurotoxicity.
Neurosurgical approaches have included stereotaxic surgery Weigel and Krauss, and high intensity focused ultrasound of various brain structures including the thalamus Martin et al.
Cingulotomy is perhaps the classic neurosurgical ablative technique reported to provide pain control Wilkinson et al. Following cingulotomy for a non-pain disorder , pain affect in response to noxious heat and cold was altered Davis et al. In patients undergoing cingulotomy, microelectode single unit measures of cingulate neurons following reward-based stimuli revealed decrease in neuronal activity predicting movement. However, following ablation, patients made more errors Williams et al.
Morphine has been postulated to have a similar effect on modulation of affect by the cingulate cortex LaGraize et al. Clearly any lesion of the brain will alter the dynamics of brain function both at a local brain region and at a systems level through afferent and efferent connections.
Stimulation techniques, whether extracranial e. Although small case studies of deep brain stimulation reported successful outcomes Owen et al. A meta-analysis of deep brain stimulation for pain Bittar et al. Even higher rates of success were seen with phantom limb pain and neuropathies. To date, stimulation sites have included the ventroposterolateral thalamus and the periaqueductal grey region.
Motor cortex stimulation has been proposed for the treatment of chronic pain Lefaucheur et al. Deep brain stimulation of the subthalamic nucleus has had a landmark effect in Parkinson's disease, but its effects on pain processing also seem potentially useful.
In one study, chronic pain was reduced following deep brain stimulation, but pain sensitivity to quantitative sensory testing was unaltered Gierthmuhlen et al. In another study, early pain relief was observed in 20 of 23 patients Kim et al. Neuroimaging studies have suggested that deep brain stimulation targeting the ipsilateral posterior inferior hypothalamus might be effective for chronic cluster headache; this is now an established treatment for intractable cases Leone, ; Leone et al.
Motor cortex stimulation is another intracranial approach that has been used to treat refractory neuropathic pain Lefaucheur et al. Given the interactions between sensory and motor systems and the fact that pain may inhibit motor cortex function patients may limit their movement Farina et al.
A relatively recent approach is the use of transcranial magnetic stimulation for chronic pain Lefaucheur, , migraine Lipton and Pearlman, , spinal cord injury pain Defrin et al. Experimental use in neurological diseases including Alzheimer's disease Bentwich et al. In Parkinson's disease Baumer et al. Further studies are needed to determine its potential clinical utility in neurological diseases with pain. Specific pharmacotherapies developed for many of these neurological conditions have been assessed for their ability to treat chronic pain Finnerup et al.
The most notably successful are the anti-epilepsy drugs, but antidepressants, membrane stabilizers and opioids have also been used to treat chronic pain, with varying levels of success. Given our new understanding that alterations in grey matter volume correlate with chronic pain, recent trials have investigated drugs with the potential to modify putative underlying disease mechanisms. The major categories of drugs that have been assayed are neuroprotectants, including the excitatory neurotransmitter antagonists e.
NMDA , and agonists of inhibitory neurotransmitter systems e. Amantidine is a drug originally used in Parkinson's disease. A derivative, memantine, an NMDA antagonist, has been marketed for treatment of Alzheimer's disease and other neurodegenerative disorders Sonkusare et al. Another example, d -cycloserine, an antibiotic, is a partial agonist of the NMDA receptor. It has been used in preclinical models of CNS degeneration Ogawa et al.
Riluzole, an FDA-approved drug for the treatment of amyotrophic lateral sclerosis, can reverse pain behaviour in spinal cord injured rats Hama and Sagen, It is thought to act by inhibiting glutamate release.
Other examples of therapies directed at primary neurological disease that may be useful for pain include: It has been used in preclinical models of Huntington's disease Smith et al. It has effects in a number of preclinical CNS-related disease models including chronic pain Sweitzer and De Leo, The use of CT or MRI to direct delivery of small amounts of drugs to specific regions or nerves for pain control is gaining increased attention in preclinical models.
The amount of drug delivered is small enough that it has no systemic effect. For example, when injected into a nerve in very small quantities, adriamycin can provide pain relief through retrograde transport and killing of the dorsal root ganglion cells of the specific nerves affected Grant et al.
Another example is the use of small volumes of agents that specifically knock out C-pain fibres. One such agent is resniferatoxin, a capsaicin analogue that inactivates sensory neurons by binding to the vanilloid TRPV1 receptor and producing a calcium influx Bates et al. Although clearly not a preferred solution since it destroys the sensory neurons, targeted delivery of such an agent may be used to control pain in certain conditions, such as cancer affecting the face, when other efforts have failed.
Another possible future application of this kind of approach is the targeted treatment of schwannomas based on newly defined preclinical developments Saydam et al. A major issue in pain diagnosis and research is the lack of an objective measurement of pain. Even in patients able to report subjective pain ratings, these are clearly insufficient Victor et al.
In cases where patients cannot communicate, the problem is even more complex. There is an urgent need to develop biomarkers for pain. The search for reliable markers of chronic pain has focused on a number of approaches. Questionnaires that have been used in the evaluation of chronic pain attempt to also determine changes that occur in addition to changes in pain intensity [e.
Tools adopted from the psychiatric literature, which can be used to evaluate other dimensions of chronic pain, including quality of life, depression, anxiety, catastrophizing and drug-abuse potential, are being included in chronic pain evaluation because of the multi-dimensional nature of the condition Haythornthwaite, These neurophysiological approaches aim to provide a differentiated assessment based on underlying pathophysiology that may include measures of sensitization, abnormal fibre type, sensory loss etc.
Few of these assessment tools are routinely used in current clinical settings. Aside from diffusion tensor imaging that is currently employed in many institutions to evaluate alterations in white matter integrity, two current imaging technologies may soon be in the clinic for evaluation of patients with pain. The first is measures of grey matter and the second is resting state networks. A defining article by Apkarian et al.
Since then a number of groups have reported such changes in various neurological conditions including trigeminal neuropathy DaSilva et al. While it is not yet well-understood, the finding that the grey matter changes revert towards normal with treatment is highly intriguing Rodriguez-Raecke et al.
A second approach with potential utility in the clinic is evaluation of resting state networks Greicius et al. Such networks can differ in disease states Chen et al. If methodological issues can be ironed out in terms of how best to evaluate the multiple resting state networks of health and disease, the approach is potentially of high value in the clinic as it does not require any intervention with patients during scanning procedures. Figure 5 summarizes integrative approaches for evaluating pain.
Of these, imaging has taken the stage in its ability to evaluate functional, morphological and chemical changes in disease states and provide a new window of understanding disease neurobiology related to chronic pain. The successful development of drug analgesic and disease neuropathic pain brain signatures and subsequently the validation as biomarkers would allow for objective indices for clinical drug development and for clinical practice.
Since brain action provides a basis for behaviour pain or analgesia brain imaging holds the promise of defining potential makers that would need to then be shown to be sensitive, reproducible, validated and subsequently adopted by clinicians and regulatory agencies.
A number of recent reviews have addressed the current state of imaging the brain state in pain and its potential in providing objective measures of drug and other therapeutic measures Tracey and Mantyh, ; Apkarian, ; Borsook and Becerra, Most neurologists treat patients with chronic pain, but few specialize in the discipline. In fact, when neurologists rate their preference for treating diseases, chronic pain ranks low with the exception of migraine, which ranks high Evans and Evans, The reasons for this may include: Thus, there is a high unmet need for chronic pain treatment and for more research into the underlying mechanisms of pain diseases.
With current technological advances and investigation into mechanism-based treatment approaches Finnerup, , we are at a critical juncture in pain research. However, progress will remain slow unless we fully recognize pain as a brain disease and increase the involvement of neurologists in the treatment of and research into chronic pain. The first step towards this goal is to include a comprehensive survey of pain conditions, management and research as part of the standard training of new neurologists.
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Introduction Recent advances in basic and clinical neuroscience suggest the brain plays a pivotal role in the chronic pain state. Open in a separate window. Table 1 Pain and neurological diseases. Pain present in most Aghakhani et al. Hyperaesthesia Milhorat et al. Typical neuropathic pain Hatem et al.
Often present van Doorn et al. Acute chronic neuropathic paraesthesias Numbness van Doorn et al. Mononeuropathy multiplex Verma, Understanding potential links between pain pathophysiology and neurological disease Recent advances have greatly increased our understanding of pain mechanisms.
Neurological diseases and pain This section discusses examples of neurological diseases that have pain as a co-existing or co-morbid process.
Central nervous system degenerative diseases Parkinson's disease and pain Parkinson's disease is perhaps the best example of co-morbid pain as an integral part of a neurodegenerative disease. Alzheimer's disease and pain Pain processing may be altered in dementias Scherder et al. Huntington's disease and pain The prevalence of pain in Huntington's disease is unknown.
Ataxia and pain Machado—Joseph disease is the most common spinocerebellar ataxia, also known as spinocerebellar ataxia type 3 Rub et al. Central nervous system damage Stroke It is well-documented that strokes affecting the CNS, particularly the structures along the spino-thalamocortico-tract spinothalamic tract, lateral thalamus, thalamic—parietal projections , produce central pain syndromes central post-stroke pain Bowsher et al.
Syringomyelia Syringomyelia and its sister disorder, syringobulbia, are disorders associated with Arnold—Chiari malformation Koyanagi and Houkin, or spinal cord trauma Schurch et al.
Traumatic brain injury Multiple pain syndromes have been described in different patients following traumatic brain injury with diffuse axonal injury Raghupathi and Margulies, , including neuropathic pain, central pain, and thalamic pain Formisano et al.
Tumours and pain Neurofibromatosis Neurofibromatosis is an autosomal dominant neurocutaneous disorder subdivided into neurofibromatosis 1 NF1 , neurofibromatosis 2 NF2 and schwannomatosis Lu-Emerson and Plotkin, Peripherally initiated changes in central nervous system pain processing Each of the three clinical examples presented in this section provides insight into how changes in peripheral pain pathways impact CNS pain processing.
Complex regional pain syndrome Perhaps no pain condition represents the centralization of pain more clearly than complex regional pain syndrome CRPS; Janig and Baron, ; Bruehl, Congenital insensitivity to pain This is a rare and severe autosomal recessive condition Rosemberg et al.
Brain-based restorative approaches for chronic pain Despite trials of a number of approaches, chronic pain is largely refractory to treatment. Motor training Motor and sensory systems are well integrated Flor and Diers, Central nervous system lesions—do they inform us more than they provide effective pain control? Brain stimulation Stimulation techniques, whether extracranial e. Centrally active drugs developed for neurological disease and their potential role in pain therapeutics Specific pharmacotherapies developed for many of these neurological conditions have been assessed for their ability to treat chronic pain Finnerup et al.
Smart treatments—targeting through localized delivery The use of CT or MRI to direct delivery of small amounts of drugs to specific regions or nerves for pain control is gaining increased attention in preclinical models. Smarter tools for objective diagnosis of pain A major issue in pain diagnosis and research is the lack of an objective measurement of pain. References Abbruzzese G, Berardelli A. Sensorimotor integration in movement disorders. Long-term follow-up of Chiari-related syringomyelia in adults: Fabry disease in patients with migraine with aura.
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Neurological disorders are diseases of the brain, spine and the nerves that connect them. There are more than diseases of the nervous system, such as . A: Neurological disorders are diseases of the central and peripheral nervous system. In other words, the brain, spinal cord, cranial nerves, peripheral nerves. Table A Burden of neurological disorders, in DALYs, by cause .. predicted rise in the prevalence of neurological and other chronic disorders and the dis-.