ALS – Frequently Used Terms
A
ACTIVITIES OF DAILY LIVING (ADLS)
Personal care skills required to manage one’s basic physical needs. These are bathing/grooming, dressing, eating, using the toilet, and transferring/ambulating.
ADVANCE DIRECTIVES
Legal documents that provide instructions for medical care that go into effect if you cannot communicate your wishes. Examples of advance directives for health care are the living will and durable power of attorney for health care.
ALS (AMYOTROPHIC LATERAL SCLEROSIS)
Also known as Lou Gehrig's disease, it is a neuromuscular disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement, and breathing. It is the most common motor neuron disease. The literal translation of amyotrophic is “without muscle nourishment”.
ANTIBODY
A defense protein produced by the immune system that binds to foreign molecules to find off these antigens.
ANTIGEN
A toxin or molecule that tells your body that something is foreign and induces an immune response, especially the production of antibodies.
ANTIOXIDANT
A chemical compound or substance that inhibits oxidation.
ATROPHY
The progressive loss of muscle mass, or wasting.
AXON
The long, hair-like extension of a nerve cell that carries a message to the next nerve cell.
B
Botox
A drug prepared from the bacterial Botulinum Toxin, used medically to treat certain muscular conditions. In ALS it is commonly used to treat excessive saliva or spastic muscles. It is also used cosmetically.
BRISK REFLEX
A condition that describes the deterioration of the upper motor nerve cells (neurons).
BULBAR
Bulbar ALS is the type in which onset symptoms are in the facial muscles, speech and swallowing.
C
CENTRAL NERVOUS SYSTEM (CNS)
The brain and spinal cord combined.
CEREBELLUM
The portion of the brain in the back of the head, between the cerebrum and the brain stem. It is responsible for the coordination of movement and balance.
CEREBRAL CORTEX
The outer layer of the brain, located on the top of the cerebrum. It carries out essential functions of the brain like memory, thinking, learning, reasoning, problem-solving, emotions, consciousness, and sensory functions.
CEREBRO-SPINAL FLUID
A clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord. It provides nourishment, waste removal and protection to the brain. Analysis helps to diagnose diseases and conditions affecting the brain and spinal cord.
CHROMOSOME
A visible carrier of the genetic information.
CHRONIC
Marked by long duration or frequent recurrence.
CORD BLOOD
Blood taken from the umbilical cord at the time of birth. It is rich in a variety of stem cells.
CORTICOSPINAL TRACT
The bundle of nerves that reach from the motor area of the brain to the spinal cord, connecting to the nerves that go out to control the muscles.
D
DEMENTIA
Not a disease itself, but a group of symptoms that is characterized by a decline in intellectual functioning that is severe enough to interfere with the ability to perform routine activities.
DENDRITE
Extensions from the neuron cell body that takes information to the cell body. A single nerve may possess many dendrites.
DNA
One of two types of molecules that encode genetic information
DYSARTHRIA
Impaired speech and language due to weakness or stiffness in the muscles used for speaking.
DYSPHAGIA
Difficulty in swallowing.
DYSPNEA
Shortness of breath; difficult or labored breathing
E
EDARAVONE
The generic name for Radicava
ELECTRODIAGNOSTIC TESTS
Studies including electromyography (EMG) and nerve conduction velocity ( NCV), that evaluate and diagnose disorders of the muscles and motor neurons. Electrodes are inserted into the muscle, or placed on the skin overlying a muscle or muscle group, and electrical activity and muscle response are recorded.
EMBRYONIC STEM CELLS
These are pluripotent stem cells, derived from the inner call mast of the blastocyst. These versatile cells can divide into more stem cells and become any type of cell in the body.
EXSERVAN
Riluzole, oral film. Each film tab that can dissolve on the tongue contains 50 mg of riluzole.
F
FAMILIAL ALS
A progressive neurological disease that affects more than one member of the same family. This type of ALS accounts for a very small number of people with ALS in the United States (5 to 10 percent).
FASCICULATION
Muscle twitching. When a single peripheral never is overactive it results in involuntary muscle movement.
FDA
The U.S. Food and Drug Administration (FDA) is the federal agency responsible for protecting public health by ensuring the safety, efficacy, and security of human and veterinary drugs, biological products, and medical devices; and by ensuring the safety of our nation's food supply, cosmetics, and products that emit radiation.
FLACCID MUSCLES
A type of paralysis whereby the muscles become soft and yield to passive stretching. These muscles cannot contract and become floppy and immobile.
FLEXOR MUSCLE
Any muscle that causes the bending of a limb or other body part.
FREE RADICALS
Chemicals that are highly reactive and can oxidize other molecules (i.e. Superoxide).
FRONTOTEMORAL DEMENTIA (FTD)
A group of disorders caused by progressive nerve cell loss in the brain's frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears). The first noticeable FTD symptoms are changes in personality and behavior and/or difficulties with language. A proportion of patients presenting with ALS develop FTD, with prevalence estimates varying between 5% and 22%.
G
GANGLION
A cluster of nerve cell bodies.
GENE
Genes are the basic biological units of heredity. They are composed of DNA.
GENOME
All of the genetic information; the entire genetic complement; all of the hereditary material possessed by an organism.
GLUTAMATE
An excitatory neurotransmitter with several types of receptors found throughout the central nervous system. Overstimulation of some of these receptors is thought to trigger neuronal damage.
H
HYPERREFLEXIA
Excessive response of muscle reflexes when a normal stimulus is applied.
HYPOREFLEXIA
Weak or absent muscle response when a normal stimulus is applied.
I
INCIDENCE
A measure of the probability of occurrence of a given medical condition in a population within a specified period of time. The occurrence of new cases of a condition. It is commonly measured in new cases per 100,000 of population at risk, per year.
INFLAMMATION
The reaction of a living tissue to injury or infection characterized by heat, redness, swelling, and pain.
INTRATHECAL
Injection into the innermost membrane surrounding the central nervous system. Usually done by lumbar puncture.
M
MOLECULE
The smallest unit of a substance that can exist alone and retain the character of that substance.
MOTOR NEURON
A neuron that conveys impulses initiating muscle contraction or glandular secretion.
MOTOR NEURON DISEASE (MND)
A group of disorders in which motor nerve cells (neurons) in the spinal cord and brain stem deteriorate and die. ALS is the most common motor neuron disease.
MUSCLE
The tissue of the body that works by contracting or relaxing to cause movement or power. This movement may be voluntary or involuntary. Muscles are either skeletal, cardiac or smooth.
MUTATION
A permanent change, a structural alteration, in the DNA or RNA. Mutations can be caused by many factors including environmental insults such as radiation and mutagenic chemicals. Mutations are sometimes attributed to random chance events.
MYOCLONUS
Jerking, involuntary movements of the arms and legs. May occur normally during sleep.
N
NERVES
Bundles of fibers that carry electrical impulses between the brain and the rest of the body. This cable network helps you to feel sensations and/or to move your muscles. Some also maintain certain autonomic functions like breathing, sweating, or digesting food.
NERVOUS SYSTEM
The body system includes the brain, spinal cord, and a complex network of nerves. It allows messages to be sent back and forth between the brain and the body.
NEUROLOGIST
A physician who specializes in the nervous system and its disorders.
NEUROLOGY
The medical science that deals with the nervous system and disorders affecting it.
NEUROMUSCULAR
Relating to nerves and muscles.
NEURONS
The basic functional units of the nervous system. The general electrical signals (action potentials) that allow them to quickly transmit information over long axons to other neurons and a host of dendrites that deliver incoming signals.
NEURONAL RECEPTORS
Proteins that transmit the actions of neurotransmitters, enabling cell-to-cell communications within the nervous system.
NEUROPROTECTIVE
Agent providing protection to the nerve cells against damage, degeneration or impairment of function.
NEUROREGENERATIVE
Agent protecting the nerve cells against damage, degeneration, or impairment of function.
NEUROSCIENCE
Neuroscience is the scientific study of the nervous system, its functions, and disorders. It is a multidisciplinary science that combines physiology, anatomy, molecular biology, and developmental biology.
NEUROTRANSMITTERS
Chemical substances that carry impulses from one nerve cell to another are found in the space (synapse) that separates the transmitting neuron's terminal (axon) from the receiving neuron's terminal (dendrite).
O
ONSET
The time of appearance of the first symptoms of a condition, before seeking diagnosis.
OXIDATIVE STRESS
A phenomenon caused by an imbalance between production and accumulation of oxygen reactive species in cells and tissues and the ability of a biological system to detoxify these reactive products.
P
PALS
Person with ALS.
PALSY
Paralysis of a muscle or group of muscles.
PEG TUBE
Percutaneous endoscopic gastrostomy tube - a feeding tube placed by a gastroenterologist.
PERISTALSIS
Intestinal motility characterized by radially symmetrical contraction and relaxation of muscles that propagate in a wave down the intestinal tract.
PHARMACOKINETICS
A branch of pharmacology dedicated to describing how the body affects a specific substance after administration.
PHENOTYPE
An individual’s observable traits. A person’s phenotype is determined by both their genomic makeup (genotype) and environmental factors.
PLURIPOTENT STEM CELLS
A particularly potent type of stem cell that can form all three of the basic body layers (ectoderm/endoderm/mesoderm) and even germ cells. They are either derived from embryonic tissue or induced through reprogrammed adult tissue.
PRIMARY LATERAL SCLEROSIS (PLS)
A slowly progressive neuromuscular disease characterized by deterioration of upper motor neurons exclusively.
PROGRESSIVE BULBAR PALSY (PBP)
A motor neuron disorder involving deterioration of lower motor neurons. It begins with difficulties with speech, swallowing, and chewing.
PROGRESSIVE MUSCULAR ATROPHY (PMA)
A progressive neurological disease in which the lower motor nerve cells (neurons) deteriorate.
PROTEIN
Proteins are large molecules required for the structure, function, and regulation of the body's cells, tissues, and organs.
PROTOCOL
A precise and detailed plan for the study of a biomedical problem or a regimen of an experimental therapy.
PSEUDOBULBAR AFFECT (PBA)
Also known as emotional lability, it is a condition that affects some people with ALS and some other neurological problems. It is characterized by uncontrolled displays of laughing or crying.
PYRAMIDAL PATHWAY
A collection of nerve tracts that travel from the cerebral cortex through the pyramid of the medulla oblongata in the brainstem to the spinal cord. Within the pyramid of the medulla, fibers cross from one side of the brain to the opposite side of the spinal cord; the pyramidal pathway is intact in Parkinson's disease.
R
RADICAVA
Brand name of edaravone. It is a free-radical scavenger that can help remove reactive molecules which could lead to oxidative stress. Studies indicate that this drug could thereby slow ALS progression.
RANGE OF MOTION
Refers to how far you can move or stretch a part of the body, such as a joint or a muscle.
RELYVRIO
FDA approved drug to help slow the progression of ALS. Its two compounds are broadly expected to work together to prevent nerve cell death by blocking stress signals within two cellular compartments, specifically mitochondria and the endoplasmic reticulum. The combination drug components are sodium phenylbutyrate and taurursodiol.
RIGIDITY
Spasticity, or stiffness of a muscle.
RILUTEK
A brand name of riluzole.
RILUTEK
The first FDA approved medication to slow the progression of ALS, but blocking the release of glutamate at the neuromuscular junction.
RNA
Ribonucleic acid (RNA) is a molecule that is present in the majority of living organisms and viruses. It is made up of nucleotides, which are ribose sugars attached to nitrogenous bases and phosphate groups. The nitrogenous bases include adenine, guanine, uracil, and cytosine.
ROUTES OF ADMINISTRATION
The location a drug is administered, such as oral, intravenous, intramuscular, sublingual, subcutaneous, enteral, rectal or parenteral
S
SCLEROSIS
Hardening
SIALORRHEA
Hypersalivation or excessive drooling.
SIDE EFFECT
A secondary, typically undesirable effect of a drug or medical treatment.
SOD (SUPEROXIDE DISMUTASE)
An antioxidant enzyme found in all living cells.
SPASM
A sudden voluntary muscular contraction.
SPINAL CORD
An extension of the central nervous system (CNS), which consists of the brain and spinal cord. The spinal cord begins at the bottom of the brain stem (at the area called the medulla oblongata) and ends in the lower back, as it tapers to form a cone called the conus medullaris.
SPINAL MUSCULAR ATROPHY (SMA)
Spinal muscular atrophy (SMA) refers to a group of hereditary diseases that can damage and kill specialized nerve cells in the brain and spinal cord (motor neurons). Motor neurons control movement in the arms, legs, face, chest, throat, and tongue, as well as skeletal muscle activity, such as speaking, walking, swallowing, and breathing.
SPORADIC
Occurring at irregular intervals or as isolated incidents. In ALS, the disease seems to occur at random with no associated risk factors and no family history of the disease. 90-95% of ALS cases are considered to be sporadic.
STEM CELLS
Cells with the potential to develop into many different types of cells in the body. They serve as a repair system for the body.
SYNAPSE
The site of transmission of electric nerve impulses between two nerve cells (neurons) or between a neuron and a gland or muscle cell (effector).
T
THERAPEUTIC INDEX
A ratio that compares the blood concentration at which a drug becomes toxic and the concentration at which the drug is effective. The larger the therapeutic index (TI), the safer the drug is.
THROMBUS
A blood clot
TIGLUTIK
Riluzole, in liquid suspension form, can be taken orally or via a PEG tube.
TOXICITY
The ability of a substance to cause harmful health effects.
TOXIN
A naturally occurring organic poison produced by the metabolic activities of living cells or organisms.
TREATMENT
Something that health care providers do for their patients to control a health problem, lessen its symptoms, or clear it up. Treatments can include medicine, therapy, surgery, or other approaches.
TREMOR
A rhythmical shaking of a limb, head, mouth, tongue or other part of the body that is involuntary in nature.
U
UPPER MOTOR NEURONS
Neurons originating in the cerebral cortex and travel down to the brain stem or spinal cord.
V
VACCINE
A biological preparation that provides active acquired immunity to a particular infectious or malignant disease.