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Hypotonia
Classification and external resources
ICD-10 P94.2, R27.
ICD-9 781.3
DiseasesDB 21417
MedlinePlus 003298
MeSH D009123

Hypotonia is a condition of abnormally low muscle tone (the amount of tension or resistance to movement in a muscle), often involving reduced muscle strength. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings P00-P96 - Certain conditions originating in the Perinatal period (P00-P04 Fetus and newborn affected by maternal factors and by complications of pregnancy labour and R00-R69 - Symptoms and Signs (R00-R09 Circulatory and Respiratory systems ( Abnormalities of Heart beat The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Muscle (from Latin musculus, diminutive of mus "mouse" is contractile tissue of the body and is derived from the Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength. Motor nerves allow the Brain to stimulate muscle contraction A motor Nerve is an Efferent nerve that exclusively contains the Axons of somatic The brain is the center of the Nervous system in animals All Vertebrates and the majority of Invertebrates have a brain Recognizing hypotonia, even in early infancy, is usually relatively straightforward, but diagnosing the underlying cause can be difficult and often unsuccessful. Diagnosis is the identification by Process of elimination, of the nature of anything The long-term effects of hypotonia on a child's development and later life depend primarily on the severity of the muscle weakness and the nature of the cause. Some disorders have a specific treatment but the principal treatment for most hypotonia of idiopathic or neurologic cause is physical therapy to help the person compensate for the neuromuscular disability.

Contents

Etiology of hypotonia

Some conditions known to cause hypotonia include:

Congenital - i. A congenital disorder is a disease or disorder that is present at birth e. present from birth (including genetic disorders presenting within 6 months)

Acquired - i. A genetic disorder is a condition caused by abnormalities in Genes or Chromosomes While some diseases such as Cancer, are due to genetic abnormalities acquired Down syndrome, Down's syndrome, or trisomy 21 is a Chromosomal disorder caused by the presence of all or part of an extra 21st chromosome. 3-Methylcrotonyl-CoA carboxylase deficiency ( 3MCC deficiency) also known as 3-Methylcrotonylglycinuria type 1 or BMCC deficiency is an inherited disorder Achondroplasia is a type of Autosomal dominant Genetic disorder that is a common cause of Dwarfism. Aicardi syndrome is a rare genetic malformation syndrome characterized by the partial or complete absence of a key structure in the brain called the corpus callosum Canavan disease, also called Canavan-Van Bogaert-Bertrand disease, aspartoacylase deficiency or aminoacylase 2 deficiency, is an autosomal Centronuclear myopathies (CNM are a group of Congenital Myopathies where cell nuclei are abnormally located in skeletal muscle cells Central core disease (CCD is an Autosomal Dominant Congenital myopathy (inborn muscle disorder Familial dysautonomia, or FD, sometimes called Riley-Day syndrome is a disorder of the Autonomic nervous system which affects the development and survival Spinal Muscular Atrophy ( SMA) is a term applied to a number of different disorders all having in common a genetic cause and the manifestation of weakness due to loss Werdnig-Hoffman disease (also known as "Severe infantile spinal muscular atrophy" or "spinal muscular atrophy type I" is an Autosomal recessive Krabbe disease (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare often fatal degenerative disorder that affects the Leigh's disease, a form of Leigh syndrome, also known as Subacute Necrotizing Encephalomyelopathy (SNEM is a rare neurometabolic disorder that affects the central Marfan syndrome (or Marfan's syndrome is a genetic disorder of the Connective tissue. Menkes disease (also called the kinky hair disease or Menkes kinky hair syndrome) is a disorder that affects Copper levels in the body Methylmalonic acidemia (MMA also called methylmalonic aciduria, is an Autosomal Recessive Metabolic disorder. Myotonic dystrophy (DM is a chronic, slowly progressing highly variable inherited multisystemic Disease that can manifest at any age from birth to old age Prader-Willi syndrome (abbreviated PWS) is a very rare Genetic disorder, in which seven genes (or some subset thereof on chromosome 15 are missing or unexpressed Tay-Sachs disease (abbreviated TSD, also known as GM2 Gangliosidosis, Hexosaminidase A deficiency or Sphingolipidosis) is a Genetic Patau syndrome, also known as trisomy 13, is a chromosomal abnormality a Syndrome in which a patient has an additional chromosome 13 due 22q13 Deletion Syndrome (spoken as twenty two q one three) also known as Phelan-McDermid Syndrome, is a genetic disorder caused by a Microdeletion on Developmental disability is a term used to describe life-long disabilities attributable to mental and/or physical or combination of mental and physical impairments Ataxia (from Greek α- as a negative prefix + -τάξις, meaning "lack of order" is a neurological sign and symptom consisting Developmental dyspraxia is one or all of a Heterogeneous range of Development disorders affecting the initiation organization and performance of action Hypothyroidism is the disease state in humans and animals caused by insufficient production of Thyroid hormone by the Thyroid gland. Cerebral palsy ( CP) is an Umbrella term encompassing a group of non-progressive, non- contagious conditions that cause Physical disability Teratology stems from the Greek ( Genitive) meaning monster, or marvel and, meaning word, speech. The benzodiazepines (pronounced, often abbreviated to "benzos") are a class of Psychoactive drugs with varying Hypnotic An acquired disorder is a Medical condition which develops post- Fetally in contrast with a Congenital disorder, which is present at Birth e. onset occurs after birth

Signs and objective manifestations

Hypotonic patients may display a variety of objective manifestations that indicate decreased muscle tone. Muscular dystrophy (MD refers to a group of genetic, Hereditary Muscle diseases that cause progressive muscle weakness Myotonic dystrophy (DM is a chronic, slowly progressing highly variable inherited multisystemic Disease that can manifest at any age from birth to old age Metachromatic leukodystrophy ( MLD, also called Arylsulfatase A deficiency) is the most common form of a family of genetic diseases known as the Leukodystrophies An infection is the detrimental Colonization of a host Organism by a foreign Species. Encephalitis is an acute Inflammation of the Brain, commonly caused by a viral Infection. Guillain-Barré syndrome ( GBS) (in French ɡilɛ̃ baˈʁe in English ˈɡiːlæn ˈbɑreɪ /ɡiːˈæn bəˈreɪ/ etc Botulism ( Latin, botulus, "sausage" is a rare but serious Paralytic illness caused by Botulin Toxin. Meningitis is Inflammation of the protective membranes covering the Brain and Spinal cord, known collectively as the Meninges. Poliomyelitis, often called polio or infantile paralysis, is an acute viral Infectious disease spread from person to person primarily via Sepsis is a serious medical condition characterized by a whole-body inflammatory state (called a Systemic inflammatory response syndrome or SIRS caused Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues Myasthenia gravis (literally "serious muscle-weakness" from Greek μύς "muscle" "weakness" and Latin gravis Coeliac Inborn errors of metabolism comprise a large class of genetic Diseases involving disorders of Metabolism. Vitamin poisoning, or hypervitaminosis, refers to a condition of high storage levels of Vitamins which can lead to Toxic Symptoms The medical Kernicterus is damage to the brain centers of infants caused by increased levels of unconjugated-indirect Bilirubin which is free (not bound to albumin Rickets is a softening of the bones in children potentially leading to fractures and deformity Traumatic brain injury (TBI also called intracranial injury, occurs when Physical trauma injures the Brain. Shaken baby syndrome ( SBS) is a form of Child abuse that occurs when an abuser violently shakes an infant or small child creating a whiplash -type In Vertebrates the central nervous system ( CNS) is the part of the Nervous system which is enclosed in the Meninges. The cerebellum ( Latin: "little brain" is a region of the Brain that plays an important role in the integration of sensory perception Hypothyroidism is the disease state in humans and animals caused by insufficient production of Thyroid hormone by the Thyroid gland. Motor skills delay is often observed, along with hypermobile or hyperflexible joints, drooling and speech difficulties, poor reflexes, decreased strength, decreased activity tolerance, rounded shoulder posture, with leaning onto supports, and poor attention and motivation. The extent and occurrence of specific objective manifestations depends upon the age of the patient, the severity of the hypotonia, the specific muscles affected, and sometimes the underlying cause. For instance, some hypotonics may experience constipation, while others have no bowel problems.

Floppy infant syndrome

Since hypotonia is most often diagnosed during infancy, it is also known as "floppy infant syndrome" or "infantile hypotonia. " Infants who suffer from hypotonia are often described as feeling and appearing as though they are "rag dolls," "pillowcases of pudding'" or a "sack of jello," easily slipping through one's hands. This image demonstrates the floppiness of a hypotonic infant. They are unable to maintain flexed ligaments, and are able to extend them beyond normal lengths. In Anatomy, the term ligament is used to denote three different types of structures Fibrous tissue that connects Bones to other bones Often, the movement of the head is uncontrollable, not in the sense of spasmatic movement, but chronic ataxia. In Anatomy, the head of an Animal is the Rostral part (from Anatomical position that usually comprises the Brain, Eyes In Medicine, a chronic disease is a Disease that is long-lasting or recurrent Ataxia (from Greek α- as a negative prefix + -τάξις, meaning "lack of order" is a neurological sign and symptom consisting Hypotonic infants often have difficulty feeding, as their mouth muscles cannot maintain a proper suck-swallow pattern, or a good breastfeeding latch. Breastfeeding is the feeding of an Infant or young Child with Breast milk directly from human Breasts, not from a Baby bottle or other

Developmental delay

Children with normal muscle tone are expected to achieve certain physical abilities within an average timeframe after birth. Most low-tone infants have delayed developmental milestones, but the length of delay can vary widely. Motor skills are particularly susceptible to the low-tone disability. A motor skill is generally agreed as a learned skill that involves voluntary movement to complete a task They can be divided into two areas, gross motor skills, and fine motor skills, both of which are affected. Fine motor skills can be defined as coordination of small Muscle movements which occur e Hypotonic infants are late in lifting their heads while lying on their stomachs, rolling over, lifting themselves into a sitting position, remaining seated without falling over, balancing, crawling, and walking. Fine motor skills delays occur in grasping a toy or finger, transferring a small object from hand to hand, pointing out objects, following movement with the eyes, and self feeding.

Speech difficulties can result from hypotonia. Low-tone children learn to speak later than their peers, even if they appear to understand a large vocabulary, or can obey simple commands. Difficulties with muscles in the mouth and jaw can inhibit proper pronunciation, and discourage experimentation with word combination and sentence-forming. Since the hypotonic condition is actually an objective manifestation of some underlying disorder, it can be difficult to determine whether speech delays are a result of poor muscle tone, or some other neurological condition, such as mental retardation, that may be associated with the cause of hypotonia. Mental retardation is a generalized triarchic disorder characterized by subaverage cognitive functioning and deficits in two or more adaptive behaviors with onset before the age

Muscle tone vs. muscle strength

The low muscle tone associated with hypotonia must not be confused with low muscle strength. In body building, good muscle tone is equated with good physical condition, with taut muscles, and a lean appearance, whereas an out-of-shape, overweight individual with fleshy muscles is said to have "poor tone. Bodybuilding is the process of maximizing Muscle hypertrophy. Overweight is generally defined as having more body fat (adipose tissue than is optimally Healthy Being overweight is a common condition especially where " Neurologically, however, muscle tone cannot be changed under voluntary control, regardless of exercise and diet.

In an article by Diane E Gagnon, M. Ed. , PT,[3] she explains

"True muscle tone is the inherent ability of the muscle to respond to a stretch. In Physiology, Medicine, and Anatomy, muscle tone (aka residual muscle tension or tonus) is the continuous and passive partial The term can also refer to the stretching of Canvas on a frame. For example, if you quickly straighten the flexed elbow of an unsuspecting child with normal tone, the biceps will quickly contract in response (automatic protection against possible injury). When the perceived danger has passed, which the brain figures out really quickly once the stimulus is removed, the muscle then relaxes, and returns to its normal resting state.
". . . The child with low tone has muscles that are slow to initiate a muscle contraction, contract very slowly in response to a stimulus, and cannot maintain a contraction for as long as his 'normal' peers. A muscles contraction (also known as a muscle twitch or simply twitch) occurs when a Muscle fibre generates tension through the action of Actin Because these low-toned muscles do not fully contract before they again relax (muscle accommodates to the stimulus and so shuts down again), they remain loose and very stretchy, never realising their full potential of maintaining a muscle contraction over time. "

Diagnosis

"A diagnosis of hypotonia is sometimes considered a form of muscular dystrophy or cerebral palsy, depending on the symptoms and the doctor. If the cause of the hypotonia is thought to lie in the brain, then it might be classified as a cerebral palsy. If the cause seems to be in the muscles, it might be classified as a muscular dystrophy, even though most forms of hypotonia are not seriously dystrophic. If the cause is thought to be in the nerves, it could be classified as either or neither. In any case, hypotonia is rarely an actual muscular dystrophy or cerebral palsy, and is often not classified as either one, or anything at all for that matter. "[4]

Diagnosing a patient includes obtaining family medical history and a physical examination, and may include such additional tests as computerized tomography (CT) scans, magnetic resonance imaging (MRI) scans, electroencephalogram (EEG), blood tests, genetic testing (such as chromosome karyotyping and tests for specific gene abnormalities), spinal taps, electromyography muscle tests, or muscle and nerve biopsy. Computed tomography (CT is a Medical imaging method employing Tomography. A blood test is a laboratory analysis performed on a Blood sample that is usually extracted from a Vein in the arm using a needle, or via Genetic testing allows the genetic Diagnosis of vulnerabilities to inherited Diseases, and can also be used to determine a person's Ancestry. A karyotype is the characteristic Chromosome complement of a Eukaryote Species. In Medicine, a lumbar puncture (colloquially known as a spinal tap) is a Diagnostic and at times therapeutic procedure that is performed in order Electromyography (EMG is a technique for evaluating and recording the activation signal of muscles A biopsy (in Greek: βίος life and όψη look/appearance is a Medical test involving the removal of cells or tissues

Mild or benign hypotonia is often diagnosed by physical and occupational therapists through a series of exercises designed to assess developmental progress, or observation of physical interactions. Since a hypotonic child has difficulty deciphering his spatial location, he may have some recognizable coping mechanisms, such as locking the knees while attempting to walk. A common sign of low-tone infants is a tendency to observe the physical activity of those around them for a long time before attempting to imitate, due to frustration over early failures. Developmental delay can indicate hypotonia.

Other names for hypotonia

Prognosis and treatment

There is currently no known treatment or cure for most (or perhaps all) causes of hypotonia, and objective manifestations can be life long. The outcome in any particular case of hypotonia depends largely on the nature of the underlying disease. In some cases, muscle tone improves over time, or the patient may learn or devise coping mechanisms that enable him to overcome the most disabling aspects of the disorder. However, hypotonia caused by cerebellar dysfunction or motor neuron diseases can be progressive and life-threatening.

Along with normal pediatric care, specialists who may be involved in the care of a child with hypotonia include developmental pediatricians (specialize in child development), neurologists, neonatologists (specialize in the care of newborns), geneticists, occupational therapists, physical therapists, speech therapists, orthopedists, pathologists (conduct and interpret biochemical tests and tissue analysis), and specialized nursing care.

If the underlying cause is known, treatment is tailored to the specific disease, followed by symptomatic and supportive therapy for the hypotonia. In very severe cases, treatment may be primarily supportive, such as mechanical assistance with basic life functions like breathing and feeding, physical therapy to prevent muscle atrophy and maintain joint mobility, and measures to try and prevent opportunistic infections such as pneumonia. Treatments to improve neurological status might involve such things as medication for a seizure disorder, medicines or supplements to stabilize a metabolic disorder, or surgery to help relieve the pressure from hydrocephalus (increased fluid in the brain). Hydrocephalus (pronunciation ˌhaɪˌdɹoʊˈsɛfələs is a term derived from the Greek words "hydro" meaning water and "cephalus" meaning head and this condition

For most hypotonics, the National Institute of Health recommends "physical therapy [to] improve motor control and overall body strength. "NIH" redirects here For other meanings of NIH see NIH (disambiguation. Occupational therapy to assist with fine motor skill development and hand control, and speech-language therapy can help breathing, speech, and swallowing difficulties. Therapy for infants and young children may also include sensory stimulation programs. " Ankle/foot orthoses are sometimes used for weak ankle muscles. Toddlers and children with speech difficulties may benefit greatly by using sign language. A sign language (also signed language) is a Language which instead of acoustically conveyed Sound patterns uses visually transmitted sign patterns

Breastfeeding

Low-tone infants often have difficulty feeding, especially coordinating the suck-swallow reflex required for proper breastfeeding. Breastfeeding is the feeding of an Infant or young Child with Breast milk directly from human Breasts, not from a Baby bottle or other [5] Early diagnosis of hypotonic newborns can help mothers find the support and information they need to establish a successful breastfeeding relationship. Hypotonic babies may take longer to breastfeed because of the poor timing of sucking bursts and the need for long rests. If feeding is inefficient, they will also require greater feeding frequency. A baby with low muscle tone may suck better when the head and bottom are level, indicating pillow support in the lap. If the infant tends to arch his back, it may be helpful to swaddle the child loosely with arms drawn across the chest and legs drawn up toward the belly with a rounded spine during feedings. It may be necessary to support the infant's chin with one's hand if jaw, ear, and temple movement are not observed. If the baby tolerates touch to the mouth and face, the mother might gently rub the baby's lips and the outer surface of the gums to stimulate muscle sensitivity before beginning feeding. "If the tongue does not have the tone, strength, or range of motion to lift and press the breast against the palate (roof of the mouth), the baby might compensate by pressing more with his jaws. This excessive compression is painful for the mother. Getting a deeper latch, making sure the baby is not tongue-tied, and using an asymmetrical latch to increase the amount of tongue in contact with the breast can all be helpful to reduce compression. " [6] Finally, if nursing is too frustrating and stressful for mother and child, breast milk can be expressed by use of a breast pump and fed through a bottle. A breast pump is a mechanical device (powered manually or by electricity that extracts Milk from the Breasts of a female who is Lactating.

Positions which allow for better drainage of the breast through the help of gravity or manual expressing/massage while the baby is nursing may improve milk intake at the breast. Proper latch can be taught/encouraged by reinforcing appropriate mouth/tongue movements with massage of the breast to increase flow. Taking extra time with feeds is helpful. Many low-tone babies have a hard time with the coordination of sucking, swallowing and breathing and taking frequent short breaks may be helpful.

Exclusive feeding of breastmilk, especially for infants who have frequent bouts of gagging due to poor coordination, can be helpful in avoiding some of the sequelae of aspiration of formula. Breastmilk is less irritating to the lungs and less likely to cause infection if aspirated. When weighing the benefits vs. risks of formula supplementation to encourage weight gain, the possibility of aspiration should be considered. It may be better to have a little slower gain and avoid the risk of non-breastmilk fluids being aspirated.

See also

References

  1. ^ 3-Methylcrotonyl-CoA Carboxylase Deficiency (3MCC)
  2. ^ Range of Neurologic Disorders in Patients With Celiac Disease (3MCC)
  3. ^ Tone versus Strength, by Diane E Gagnon, M.Ed., PT. Hypertonia is an Upper motor neuron dysfunction marked by an abnormal increase in tightness of Muscle tone and a reduced ability of a muscle to stretch Retrieved on 2007-06-07. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 1099 - The First Crusade: The Siege of Jerusalem begins
  4. ^ The Benign Congenital Hypotonia Site. Retrieved on 2007-06-07. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 1099 - The First Crusade: The Siege of Jerusalem begins
  5. ^ Breastfeeding an infant with neurological problems (PDF)
  6. ^ Tactile Defensiveness and Other Sensory Modulation Difficulties (with Breastfeeding) Catherine Watson Genna, BS, IBCLC, New York City, New York, USA, From: LEAVEN, Vol. 37 No. 3, June-July 2001, pp. 51-53]. Retrieved on 2007-06-07. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 1099 - The First Crusade: The Siege of Jerusalem begins

External links

Support organizations

"NIH" redirects here For other meanings of NIH see NIH (disambiguation. The National Institute of Neurological Disorders and Stroke is a part of the U

Dictionary

hypotonia

-noun

  1. An abnormal loss of muscle tone
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