Hypotonia , commonly known as floppy baby syndrome , is a state of low muscle tone (the amount of tension or resistance to stretch the muscles), often involving a decrease in muscle strength. Hypotonia is not a special medical disorder, but a potential manifestation of many different diseases and disorders that affect motor neuron control by the brain or muscle strength. Hypotonia is resistance to passive movement, while muscle weakness causes active movement disorder. Central hypothonia is derived from the central nervous system, while peripheral hypothonia is associated with problems in the spinal cord, peripheral nerves and/or skeletal muscle. Recognizing hypotonia, even early in infancy, is usually relatively easy, but diagnosing underlying causes can be difficult and often unsuccessful. The long-term effects of hypotonia on child development and subsequent life depend heavily on the severity of muscle weakness and the nature of the cause. Some disorders have specific treatment but the main treatment for most idiopathic or neurologic hypotonic causes is physical therapy, occupational therapy for remediation, and/or music therapy.
Hypotonia is thought to be associated with disturbance of afferent inputs from stretch receptors and/or lack of influence of the cerebellar facies facilitators in the system, which supply the intrafusal muscle fibers thus controlling the sensitivity of muscle spindles. On examination, reduced resistance to passive movement will be noted and the muscles may feel very soft and weak on palpation. The lack of deep tendon reflexes may also be noted. Hypotonia is a condition that can be helped by early intervention.
Video Hypotonia
Signs and symptoms
Hypotonic patients may present a variety of objective manifestations that show a decrease in muscle tone. Motor delays are often observed, along with hypermobile or hyperflexible joints, drooling and speech impairment, poor reflexes, decreased strength, decreased activity tolerance, rounded shoulder posture, with reliance on support, and poor attention. The extent and occurrence of specific objective manifestations depends on the patient's age, the severity of the hypotonia, the particular muscles involved, and sometimes the underlying cause. For example, some people with hypotonia may experience constipation, while others do not have bowel problems.
Floppy baby syndrome
The term "floppy baby syndrome" is used to describe abnormal abnormalities when the baby is born. Babies who suffer from hypotonia are often described as feelings and appear as if they are "cloth dolls". They can not keep the ligaments bent, and are able to extend them beyond the normal length. Often, head movements can not be controlled, not in the sense of spasm movement, but chronic ataxia. Hypotonic infants often have difficulty eating, because their oral muscles can not maintain proper sucking-swallowing patterns, or good breastfeeding latches.
Development delay
Children with normal muscles are expected to achieve certain physical abilities in the average time period after birth. Most babies with low tones have delayed developmental achievements, but the length of delay can vary greatly. Motor skills are particularly vulnerable to low-tone defects. They can be divided into two areas, gross motor skills, and fine motor skills, both of which are affected. A late hypotonic baby lifts the head while lying on the abdomen, rolls over, lifts up to a sitting position, sits without falling, balancing, crawling, and occasionally walking. Delays in fine motor skills occur in grasping toys or fingers, moving small objects from hand to hand, showing objects, following movements with eyes, and feeding themselves.
Difficulty speaking can occur due to hypotonia. Children with low tones learn to speak more slowly than their peers, even if they seem to understand a large vocabulary, or can obey simple commands. Difficulty with the muscles in the mouth and jaw can inhibit proper pronunciation, and prevent experiments with word combinations and sentence formation. Since hypotonic conditions are actually an objective manifestation of some underlying disorder, it can be difficult to determine whether speech delay is a result of poor muscle tone, or some other neurological condition, such as intellectual disability, which may be related to the cause of hypotonia. In addition, lower muscle tone may be caused by Mikhail-Mikhail syndrome, characterized by muscle atrophy and cerebellar ataxia due to abnormalities in the ATXN1 gene.
Muscle tone vs. muscle strength
Low muscle tone associated with hypotonia should not be confused with low muscle strength or the definition commonly used in body formation. The neurological muscle tone is a manifestation of the periodic action potential of motor neurons. Because it is an intrinsic property of the nervous system, it can not be changed through voluntary control, exercise, or diet.
- "The right muscle is the ability to attach to the muscle to respond to stretching, for example, rapidly straightening the flexed elbow in an unsuspecting child with a normal tone, will cause the biceps to contract in response (automatic protection) to possible injury) When the perceived danger has passed, (whose brain counts after the stimulus is removed), the muscles will relax and return to their normal resting state. "
- "Children with low tones have slower muscles to start muscle contractions, contract very slowly in response to stimuli, and can not sustain contractions as long as their peers 'normal'. Because these low muscles do not fully contract before they are back relaxed (the muscles accommodate the stimulus and die again), they remain loose and very elastic, never realizing their full potential of maintaining muscle contraction over time. "
Maps Hypotonia
Cause
Some conditions known to cause hypotonia include:
Congenital - a person's disease (including a genetic disorder that occurs within 6 months)
- Genetic disorders are the most common cause
- 22q13 removal syndrome a.k.a. Phelan-McDermid Syndrome
- Carboxylase deficiency 3-Methylcrotonyl-CoA
- Achondroplasia
- Aicardi's Syndrome
- Autism spectrum disorder
- Canavan's Disease
- Centronuclear myopathy (including myotubular myopathy)
- Central core disease
- CHARGE syndrome
- Cohen's Syndrome
- Costello's syndrome
- Dejerine-Sottas Disease (HMSN Type III)
- Down syndrome a.k.a. trisomy 21 - most common
- Ehlers-Danlos syndrome
- Familial familiarization (Riley-Day syndrome)
- FG syndrome
- Fragile X syndrome
- Griscelli Type 1 Syndrome (Elejalde Syndrome)
- Disorder Growth Hormone Disorder Pituitary Dwarfism
- Lack of Holocarboxylase synthase/Double carboxylase deficiency
- Krabbe's Disease
- Leigh's Disease
- Lesch-Nyhan syndrome
- Marfan syndrome
- Menkes syndrome
- Methylmalonic acidemia
- Myotonic dystrophy
- Niemann-Pick's Disease
- Nonketotic hyperglycaemia (NKH) or Glycine encephalopathy (GCE)
- Noonan Syndrome
- Neurofibromatosis
- Patau Syndrome a.k.a. trisomy 13
- Prader-Willi syndrome
- Rett syndrome
- Septo-optical dysplasia (de Morsier syndrome)
- Spinal muscular atrophy (SMA)
- Deficiency of dehydrogenase semineral dehydrogenase (SSADH)
- Tay-Sachs Disease
- Werdnig-Hoffmann syndrome - Spinal muscular atrophy with congenital degeneration of the anterior horn of the spinal cord. Autosomal recessive
- Wiedemann-Steiner syndrome
- Williams Syndrome
- Zellweger's syndrome a.k.a cerebrohepatorenal syndrome
- Developmental flaws
- Cerebellar ataxia (default)
- sensory processing interruption
- Developmental coordination disorder
- Hypothyroidism (default)
- Cerebral palsy hypotonic
- Teratogenesis from exposure in utero to Benzodiazepines
Acquired
Acquired - e.g. occurs after birth
- Genetic
- Muscular dystrophy (including Myotonic dystrophy) - most common
- Metachromatic leukodystrophy
- Rett syndrome
- Spinal muscular atrophy
- Infection
- Encephalitis
- Guillain-BarrÃÆ' Syndrome  ©
- Baby Botulism
- Meningitis
- Poliomyelitis
- Sepsis
- Poison
- Acrodynia infantil (mercury poisoning in childhood)
- Autoimmune disorders
- Myasthenia gravis - most common
- An abnormal vaccine reaction
- celiac disease
- Metabolic disorders
- Hypervitaminosis
- Kernicterus
- Rickets
- Neurological
- Traumatic brain injury, such as damage caused by a shaken baby syndrome
- Lower motor neurone lesions
- Top motor neurone lesions
- Miscellaneous
- Central nervous system dysfunction, including Cerebellar lesions and cerebral palsy
- Hypothyroidism
- Sandifer's syndrome
- Neonatal Benzodiazepine withdrawal syndrome in children born to mothers treated in late pregnancy with benzodiazepine drugs
Diagnosis
The approach to diagnosing the cause of hypotonia (like all syndromes in neurology) is to first localize. Doctors must first determine whether hypotonia is caused by muscle, neuromuscular junction, nerve, or major cause. This will narrow down the possible cause. If the cause of hypotonia is found located in the brain, then it can be classified as cerebral palsy. If the cause is localized to the muscle, it can be classified as muscular dystrophy. If the cause is suspected to be in the nerves, it is called hypothonia due to polyneuropathy. Many cases can not be diagnosed with certainty.
Diagnosis of patients includes obtaining family medical history and physical examination, and may include additional tests such as computerized tomography scans (CT), magnetic resonance imaging (MRI), electroencephalogram (EEG), blood tests, genetic testing (such as chromosome karyotyping and tests for gene abnormalities certain), spinal tap, electromyography muscle test, or muscle and nerve biopsy.
Mild or benign hyphotonics are often diagnosed by physical and occupational therapists through a series of exercises designed to assess developmental development, or observation of physical interactions. Because a hypotonic child has trouble interpreting his spatial location, he may have a recognizable coping mechanism, such as locking his knees while trying to walk. The general sign of a low-tone baby is the tendency to observe the physical activity of those around them for a long time before trying to imitate, due to frustration over initial failure. Developmental delay may show hypotonia.
Terminology
The term hypotonia comes from the Ancient Greek ??? - ( hypo - ), "below" and ????? ( tÃÆ'³nos ), from ????? ( tein? ), "to stretch". Other terms for this condition include:
Prognosis and treatment
There is currently no known cure or cure for most (or perhaps all) causes of hypotonia, and objective manifestations can last a lifetime. The results in a particular hypotonia case depend heavily on the underlying nature of the disease. In some cases, muscle tone improves over time, or patients can learn or develop coping mechanisms that allow them to address the most crippling aspects of the disorder. However, hypotonia caused by cerebellar dysfunction or motor neurone disease can be progressive and life-threatening.
Along with normal child care, specialists who may be involved in the care of a child with hypotonia include developmental pediatricians (specializing in child development), neurologists, neonatologists (specializing in newborn care), geneticists, occupational therapists, therapists physical, speech therapist, orthopedic, pathologist (conducting and interpreting biochemical and tissue analysis tests), and special care.
If underlying cause is known, treatment is tailored to specific disease, followed by symptomatic therapy and supportive for hypotonia. In very severe cases, treatment may be particularly supportive, such as mechanical assistance with basic life functions such as breathing and feeding, physical therapy to prevent muscle atrophy and maintain joint mobility, and measures to try to prevent opportunistic infections such as pneumonia. Treatment to improve neurological status may involve things like drugs for seizure disorders, drugs or supplements to stabilize metabolic disorders, or surgery to help reduce the pressure of hydrocephalus (increase fluid in the brain).
The National Institute of Neurological Disorders and Stroke states that physical therapy can improve overall motor control and body strength in individuals with hypotonia. This is essential for maintaining postural stability statically and dynamically, which is important because postural instability is a common problem in people with hypotonia. A physiotherapist may develop a patient-specific training program to optimize postural control, to improve balance and safety. To protect against postural asymmetry the use of supporting and protective devices may be necessary. Physical therapists may use neuromuscular/sensory stimulation techniques such as rapid stretching, resistance, joint approach, and tapping to improve tone by facilitating or increasing muscle contraction in patients with hypotonia. For patients who exhibit muscle weakness in addition to hypotonic reinforcement exercises that do not overload the indicated muscles. Electrical Muscle Stimulation, also known as Neuromuscular Electrical Stimulation (NMES) can also be used to "activate hypotonic muscles, increase strength, and produce movement in paralyzed legs while preventing unused atrophy (p.498)." When using NMES, it is important to ask the patient to focus on efforts to contract the stimulated muscles. Without such concentration on movement efforts, the transition to the will movement can not be done. NMES should ideally be combined with functional training activities to improve results.
Occupational therapy can help patients with increased independence with daily tasks through improved motor skills, strength, and functional resilience. Language speech therapy can help overcome difficulty breathing, talking, and/or swallowing patients. Treatment for infants and young children may also include sensory stimulation programs. "A physical therapist may recommend ankle/foot ankle to help patients compensate for weak lower leg muscles, and toddlers and children with difficulty speaking may benefit greatly by using sign language.
See also
- Hypertonia
References
Further reading
Martin C, Inman J, Kirschner A, Deming K, Gumbel R, Voelker L (2005). "Characteristics of hypotonia in children: consensus opinion of child laborers and physical therapists". Pediatric Physical Therapy . 17 (4): 275-82. doi: 10.1097/01.pep.0000186506.48500.7c. PMIDÃ, 16357683.
External links
- hypotonia in NINDS
- Hypotonia - Medline Plus
Source of the article : Wikipedia
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