EEG waveform

the electroencephalogram (EEG) is the depiction of the electrical activity occurring at the surface of the brain. This activity appears on the screen of the EEG machine as waveforms of varying frequency and amplitude measured in voltage (specifically microvoltages). EEG waveforms are generally classified according to their frequency, amplitude, and shape, as well as the sites on the scalp at which they are recorded. The most familiar classification uses EEG waveform frequency (eg, alpha, beta, theta and delta). Information about waveform frequency and shape is combined with the age of the patient, state of alertness or sleep, and location on the scalp to determine significance. Normal EEG waveforms, like many kinds of waveforms, are defined and described by their frequency, amplitude, and location.

Frequency (Hertz, Hz) is a key characteristic used to define normal or abnormal EEG rhythms. Most waves of 8 Hz and higher frequencies are normal findings in the EEG of an awake adult. Waves with a frequency of 7 Hz or less often are classified as abnormal in awake adults, although they normally can be seen in children or in adults who are asleep. In certain situations, EEG waveforms of an appropriate frequency for age and state of alertness are considered abnormal because they occur at an inappropriate scalp location or demonstrate irregularities in rhythmicity or amplitude. Some waves are recognized by their shape, scalp location or distribution, and symmetry. Certain patterns are normal at specific ages or states of alertness and sleep. The morphology of a wave may resemble specific shapes, such as vertex (V) waves seen over the vertex of the scalp in stage 2 sleep or triphasic waves that occur in the setting of various encephalopathies.

Frequency
The frequencies most brain waves range from are 0.5-500 Hz. However, the following categories of frequencies are the most clinically relevant:

Alpha waves - 8-13 Hz Beta waves - Greater than 13 Hz Theta waves - 3.5-7.5 Hz Delta waves - 3 Hz or less

Alpha waves (see Image 1)

Alpha waves generally are seen in all age groups but are most common in adults. They occur rhythmically on both sides of the head but are often slightly higher in amplitude on the nondominant side, especially in right-handed individuals. A normal alpha variant is noted when a harmonic of alpha frequency occurs in the posterior head regions. They tend to be present posteriorly more than anteriorly and are especially prominent with closed eyes and with relaxation. Alpha activity disappears normally with attention (eg, mental arithmetic, stress, opening eyes). In most instances, it is regarded as a normal waveform. An abnormal exception is alpha coma, most often caused by hypoxic-ischemic encephalopathy of destructive processes in the pons (eg, intracerebral hemorrhage). In alpha coma, alpha waves are distributed uniformly both anteriorly and posteriorly in patients who are unresponsive to stimuli.

Beta waves (see Image 1)

Beta waves are observed in all age groups. They tend to be small in amplitude and usually are symmetric and more evident anteriorly. Drugs, such as barbiturates and benzodiazepines, augment beta waves.

Theta waves (see Image 1)

Theta waves normally are seen in sleep at any age. In awake adults, these waves are abnormal if they occur in excess. Theta and delta waves are known collectively as slow waves.

Delta waves (see Image 1)

These slow waves have a frequency of 3 Hz or less. They normally are seen in deep sleep in adults as well as in infants and children. Delta waves are abnormal in the awake adult. Often, they have the largest amplitude of all waves. Delta waves can be focal (local pathology) or diffuse (generalized dysfunction).

Morphology
This section identifies some normal waveforms, including K complex, V waves, lambda waves, positive occipital sharp transients of sleep (POSTS), spindles, mu rhythm, spikes, sharp waves, and certain delta waves (polyphasic and monophasic shapes). These waves are recognized by their shape and form and secondarily by their frequency. They include waves that may be normal in some settings and abnormal in others (eg, spikes, sharp waves). K complex (see Image 2)

V waves

K complex waves are large-amplitude delta frequency waves, sometimes with a sharp apex. They can occur throughout the brain and usually are higher in amplitude and more prominent in the bifrontal regions. Usually symmetric, they occur each time the patient is aroused partially from sleep. Semiarousal often follows brief noises; with longer sounds, repeated K complexes can occur. K complexes sometimes are followed by runs of generalized rhythmic theta waves; the whole complex is termed an arousal burst.

V waves are sharp waves that occur during sleep. They are largest and most evident at the vertex bilaterally and usually symmetrically. They show phase reversal at the vertex. V waves tend to occur especially during stage 2 sleep and may be multiple. Often, they occur after sleep disturbances (eg, brief sounds) and, like K complexes, may occur during brief semiarousals. V waves are easy to recognize.

Lambda waves (see Image 3)

Lambda waves occur in the occipital regions bilaterally as positive (upgoing) waves. They are triangular in shape and generally symmetric. They occur in the awake patient and are said to be most evident when the subject stares at a blank, uniform surface. Lambda waves occur when reading and occasionally when watching TV. Morphologically, they are similar to POSTS both in form and in occipital distribution.

Positive occipital sharp transients of sleep (see Image 3)

POSTS are triangular waves that occur in the bilateral occipital regions as positive (upgoing) waves. They can be multiple and usually are symmetric. POSTS occur in sleeping patients and are said to be most evident in stage 2 of sleep, although they are not uncommon in stage 1. POSTS are similar if not identical to lambda waves both morphologically and in the occipital distribution.

Sleep spindles

Spindles are groups of waves that occur during many sleep stages but especially in stage 2. They have frequencies in the upper levels of alpha or lower levels of beta. Lasting for a second or less, they increase in amplitude initially and then decrease slowly. The waveform resembles a spindle. They usually are symmetric and are most obvious in the parasagittal regions.

Mu waves - Wicket rhythm or rhythm en arceau (see Image 4)

Mu waves are runs of rhythmic activity that have a specific shape. They are rounded in one direction with a sharp side in the other direction. Frequency is one half of the fast (beta) activity. Mu waves disappear with motor acts of the contralateral hand or arm. Unlike alpha activity, they are not blocked by eye opening. They often are asymmetric. Mu waves are seen best when the cortex is exposed or if bone defects (eg, postsurgical) are present in the skull. They tend to be more evident over the motor cortex and in the parasagittal regions.

Spikes and sharp waves

These are recognized by their height, their sharp top, and their narrow base. Spikes and sharp waves usually are abnormal. They can be normal in the following settings: o V waves of sleep in the parasagittal regions in stage 2 sleep can be normal.

o o o

Small, sharp spikes of sleep or benign epileptiform transients of sleep (BETS) are nonpathologic. They occur in the temporal regions, often switching from side to side. They do not have slow-following waves as do most of the pathologic spikes of epilepsy. Numerous artifacts resemble spikes, but they are distinguished by other waves that may be present, by observation of the patient while they are occurring, and by experience. POSTS can have a sharp contour yet be quite normal. They occur in the occipital regions bilaterally during sleep.

Benign epileptic transients of sleep (see Image 5)

These sharp, usually small waves occur on one or both sides (usually asynchronously), especially in the temporal and frontal regions. BETS are rare in children but are more frequent in adults and elderly persons. Although they can occur in epileptic patients, BETS often are seen in individuals without epilepsy and can be regarded as a probable normal variant.

Patient Education
For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Electroencephalography (EEG).

Multimedia
Media file 1: Examples of alpha, beta, theta, and delta electroencephalography frequencies.

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Media file 2: Example of a K complex.

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Media file 3: Example of either lambda or positive occipital sharp transients of sleep (POSTS).

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Media file 4: Example of mu waveforms.

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Media file 5: Example of small sharp spikes, also known as benign epileptiform transients of sleep (BETS).

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Pompe's disease

is a type __2_ glycogen storage disease.

The citric acid cycle also known as the tricarboxylic acid cycle (TCA cycle), the Krebs cycle, or the Szent-Gyrgyi-Krebs
cycle, [1][2] is a series of enzyme-catalysed chemical reactions, which is of central importance in all living cells that use oxygen as part of cellular respiration. In eukaryotic cells, the citric acid cycle occurs in the matrix of the mitochondrion. The components and reactions of the citric acid cycle were established by seminal work fromAlbert Szent-Gyrgyi and Hans Krebs. In aerobic organisms, the citric acid cycle is part of a metabolic pathway involved in the chemical conversion of carbohydrates, fats and proteins into carbon dioxide and water to generate a form of usable energy. Other relevant reactions in the pathway include those inglycolysis and pyruvate oxidation before the citric acid cycle, and oxidative phosphorylationafter it. In addition, it provides precursors for many compounds including some amino acidsand is therefore functional even in cells performing fermentation.

Products
Products of the first turn of the cycle are: one GTP (or ATP), three NADH, one QH2, two CO2. Because two acetyl-CoA molecules are produced from each glucose molecule, two cycles are required per glucose molecule. Therefore, at the end of two cycles, the products are: two GTP, six NADH, two QH2, and four CO2

glycolysis
The most common type of glycolysis is the Embden-Meyerhof-Parnas pathway, which was first discovered by Gustav Embden, Otto Meyerhof andJakub Karol Parnas. Glycolysis also refers to other pathways, such as the EntnerDoudoroff pathway and various heterofermentative and homofermentative pathways. However, the discussion here will be limited to the Embden-Meyerhof pathway.

Glycolysis in disease
[edit]Genetic

diseases

Glycolytic mutations are generally rare due to importance of the metabolic pathway, this means that the majority of occurring mutations result in an inability for the cell to respire, and therefore cause the death of the cell at an early stage. However some mutations are seen.

This section requires expansion.

[edit]Cancer Malignant rapidly-growing tumor cells typically have glycolytic rates that are up to 200 times higher than those of their normal tissues of origin. This phenomenon was first described in 1930 by Otto Warburg and is referred to as the Warburg effect. The Warburg hypothesis claims that cancer is primarily caused by dysfunctionality in mitochondrial metabolism, rather than because of uncontrolled growth of cells. A number of theories have been advanced to explain the Warburg effect. This high glycolysis rate has important medical applications, as high aerobic glycolysis by malignant tumors is utilized clinically to diagnose and monitor treatment responses of cancers by imaging uptake of 2-18F-2-deoxyglucose (FDG) (a radioactive modified hexokinase substrate) withpositron emission tomography (PET).[12][13] There is ongoing research to affect mitochondrial metabolism and treat cancer by reducing glycolysis and thus starving cancerous cells in various new ways, including a ketogenic diet. [edit]Alzheimer's

disease

Disfunctioning glycolysis or glucose metabolism in fronto-temporo-parietal and cingulate cortices has been associated with Alzheimer's disease [14], probably due to the decreased amyloid (1-42) (A42) and increased tau, phosphorylated tau in cerebrospinal fluid (CSF) [15]

Krabbe disease
[edit]Causes

(also known as globoid cell leukodystrophy[1] or galactosylceramide lipidosis) is a rare, often

fatal degenerative disorder that affects the myelin sheath of the nervous system. This condition is inherited in an autosomal recessive pattern. The disease is named for the Danish neurologistKnud Haraldsen Krabbe.[2]

Krabbe disease is caused by mutations in the GALC gene, which causes a deficiency of an enzyme called galactocerebrosidase. The build up of unmetabolized lipids affects the growth of the nerve's protective myelin sheath (the covering that insulates many nerves) and causes severe degeneration of motor skills. As part of a group of disorders known as leukodystrophies, Krabbe disease results from the imperfect growth and development of myelin. [edit]Symptoms

Infants with Krabbe disease are normal at birth. Symptoms begin between the ages of 3 and 6 months with irritability, fevers, limb stiffness, seizures, feeding difficulties, vomiting, and slowing of mental and motor development. In the first stages of the disease, doctors often mistake the symptoms for those of cerebral palsy. Other symptoms include muscle weakness, spasticity, deafness, optic atrophy and blindness, paralysis, and difficulty when swallowing. Prolonged weight loss may also occur. There are also juvenile- and adult-onset cases of Krabbe disease, which have similar symptoms but slower progression.

Fabry disease

(also known as Fabry's disease, Anderson-Fabry disease, angiokeratoma corporis

diffusum and alpha-galactosidase A deficiency) is a rare X-linked recessive (inherited) lysosomal storage disease, which can cause a wide range of systemic symptoms.[1] The disease is named after one of its discoverers, Johannes Fabry.[2]

Symptoms

Symptoms are typically first experienced in early childhood and can be very difficult to understand; the rarity of Fabry disease to many clinicians sometimes leads to misdiagnoses. Manifestations of the disease usually increase in number and severity as an individual ages. Renal involvement Kidney complications are a common and serious effect of the disease; renal insufficiency and renal failure may worsen throughout life. Proteinuria(which causes foamy urine) is often the first sign of kidney involvement. End stage renal failure in males can typically occur in the third decade of life, and is a common cause of death due to the disease. Cardiac manifestations Cardiac complications occur when glycolipids build up in different heart cells; heart related effects worsen with age and may lead to increased risk of heart disease. Hypertension (high blood pressure) and cardiomyopathy are commonly observed. Dermatological manifestations Angiokeratomas (tiny, painless papules that can appear on any region of the body, but are predominant on the thighs, around the belly-button, buttocks, lower abdomen, and groin) are a common symptom. Anhidrosis (lack of sweating) is a common symptom, and less commonly hyperhidrosis (excessive sweating). Additionally, patients can exhibit Raynaud's disease-like symptoms with neuropathy (in particular, burning extremity pain). Ocular manifestations

Cosmetic ocular involvement may be present showing cornea verticillata (also known as vortex keratopathy), i.e. clouding of the corneas. Keratopathy may be the presenting feature in asymptomatic carriers, and must be differentiated from other causes of vortex keratopathy (e.g. drug deposition in the cornea). This clouding does not affect vision. Other ocular findings that can be seen include conjunctival aneurysms, posterior spoke-like cataracts, papilloedema, macular edema, optic atrophyand retinal vascular dilation. Other manifestations; Fatigue, neuropathy (in particular, burning extremity pain), cerebrovascular effects leading to an increased risk of stroke, tinnitus (ringing in the ears),vertigo, nausea, inability to gain weight, and diarrhea are other common symptoms.

Hurler syndrome,

also known as mucopolysaccharidosis type I (MPS I), Hurler's disease, is a genetic

disorder that results in the buildup of mucopolysaccharides due to a deficiency of alpha-L iduronidase, an enzyme responsible for the degradation ofmucopolysaccharides in lysosomes.[1]:544 Without this enzyme, a buildup of heparan sulfate and dermatan sulfate occurs in the body.Symptoms appear during childhood and early death can occur due to organ damage. MPS I is divided into three subtypes based on severity of symptoms. All three types result from an absence of, or insufficient levels of, the enzyme -Liduronidase. MPS I H or Hurler syndrome is the most severe of the MPS I subtypes. The other two types are MPS I S or Scheie syndrome and MPS I H-S or Hurler-Scheie syndrome. Hurler syndrome is often classified as a lysosomal storage disease, and is clinically related to Hunter Syndrome.[citation needed] Hunter is X-linked while Hurler is autosomal recessive. It is named for Gertrud Hurler.[2][3] The condition is marked by progressive deterioration, hepatosplenomegaly, dwarfism and unique facial features. There is a progressive mental retardation, with death frequently occurring by the age of 10 years. Developmental delay is evident by the end of the first year, and patients usually stop developing between ages 2 and 4. This is followed by progressive mental decline and loss of physical skills. Language may be limited due to hearing loss and an enlarged tongue. In time, the clear layers of the cornea become clouded and retinas may begin to degenerate. Carpal tunnel syndrome (or similar compression of nerveselsewhere in the body) and restricted joint movement are common. Affected children may be large at birth and appear normal but may have inguinal (in the groin) or umbilical (where the umbilical cord passes through the abdomen) hernias. Growth in height may be initially faster than normal, then begins to slow before the end of the first year and often ends around age 3. Many children develop a short body trunk and a maximum stature of less than 4 feet. Distinct facial features (including flat face, depressed nasal bridge, and bulging forehead) become more evident in the second year. By age 2, the ribs have widened and are oar-shaped. The liver, spleen and heart are often enlarged. Children may experience noisy breathing and recurring upper respiratory tract and ear infections. Feeding may be difficult for some children, and many experience periodic bowel problems. Children with Hurler syndrome often die before age 10 from obstructive airway disease, respiratory infections, or cardiac complications.

Hunter syndrome,

or mucopolysaccharidosis II (MPS II), is a serious genetic disorder that primarily affects

males. It interferes with the body's ability to break down and recycle specificmucopolysaccharides, also known as glycosaminoglycans or GAG. Hunter syndrome is one of several related lysosomal storage diseases. In Hunter syndrome, GAG builds up in cells throughout the body due to a deficiency or absence of the enzyme iduronate-2-sulfatase (I2S). This buildup interferes with the way certain cells and organs in the body function and leads to a number of serious symptoms. As the buildup of GAG continues throughout the cells of the body, signs of Hunter syndrome become more visible. Physical manifestations for some people with Hunter syndrome include distinct facial features and large head. In some cases of Hunter syndrome, central nervous systeminvolvement leads to developmental delays and nervous system problems. Not all people with Hunter syndrome are affected by the disease in exactly the same way, and the rate of symptom progression varies widely. However, Hunter syndrome is always severe, progressive, and life-limiting.

A pancreaticoduodenectomy,

pancreatoduodenectomy,[1] Whipple procedure, or Kausch-

Whipple procedure, is a major surgical operation involving the pancreas, duodenum, and other organs. This operation is performed to treat cancerous tumours on the head of thepancreas, malignant tumors involving common bile duct or duodenum near the pancreas.

Zollinger-Ellison syndrome is a disorder where increased levels of the hormone gastrin are produced, causing the stomach to
produce excess hydrochloric acid. Often the cause is a tumor (gastrinoma) of the duodenum or pancreas producing the hormone gastrin. Gastrin then causes an excessive production of acid which can lead to peptic ulcers in almost 95% of patients.