Showing posts with label HYPOTONIA. Show all posts
Showing posts with label HYPOTONIA. Show all posts

Monday, 9 January 2012

IPHREHAB : HYPOTONIA MANIFESTATION

IPHREHAB :

HYPOTONIA MANIFESTATION


Signs and objective manifestations
Hypotonic patients may display a variety of objective manifestations that indicate decreased muscle tone.
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.

§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" or a "sack of jello," easily slipping through one's hands.
§They are unable to maintain flexed ligaments, and are able to extend them beyond normal lengths. Often, the movement of the head is uncontrollable.
§Hypotonic infants often have difficulty feeding, as their mouth muscles cannot maintain a proper suck-swallow pattern, or a good breastfeeding latch.

Developmental delay
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.
Gross motor skills, and fine motor skills, both are affected. 
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.

Diagnosis
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.

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

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. 
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). 

Breast Feeding
Low-tone infants often have difficulty feeding, especially coordinating the suck-swallow reflex required for proper breastfeeding.
Take longer to breastfeed because of the poor timing of sucking bursts and the need for long rests. 
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 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.

IPHREHAB : HYPOTONIA AND ETIOLOGY

IPHREHAB

HYPOTONIA


Disorders of muscle tone Hypotonia

  • Hypotonia is a condition of abnormally low muscle tone, often involving reduced muscle strength.
  • 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. 
  • Recognizing hypotonia, even in early infancy, is usually relatively straightforward, but diagnosing the underlying cause can be difficult and often unsuccessful.


§
  • 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 neurological cause is physical therapy to help the person compensate for the neuromuscular disability .
Etiology of Hypotonia
§
  • Congenital - i.e. present from birth (presenting within 6 months) 
§
  • Genetic disorders are the most common cause
Down's syndrome - most common
3-Methylcrotonyl-CoA carboxylase deficiency
Achondroplasia
Aicardi syndrome
Canavan disease
Centronuclear myopathy
Familial dysautonomia
Infantile spinal muscular atrophy
Krabbe disease

Leigh's disease
Marfan's syndrome
Menkes syndrome
Methylmalonic acidemia
Nonketotic Hyperglycinemia (NKH) or Glycine Encephalopathy (GCE)
Prader-Willi syndrome
Tay-Sachs disease
Trisomy 13
22q13 deletion syndrome
FG Syndrome

Developmental Disability
Cerebellar ataxia (congenital) 
Dysfunction in Sensory Integration (DSI) 
Developmental Dyspraxia 
Hypothyroidism (congenital) 
Hypotonic Cerebral palsy 
Teratogenesis from in utero exposure to Benzodiazepines 

Acquired
Genetic 
  1. Muscular dystrophy (including Myotonic dystrophy) - most common 
  2. Metachromatic leukodystrophy 
  • Infections 
Encephalitis
Guillian-Barre syndrome
Infant botulism
Meningitis
Poliomyelitis
Sepsis
§
  • Autoimmunity disorders  
Myasthenia gravis- most common
Abnormal vaccine reaction
§
  • Metabolic disorder
Hypervitaminosis
Kernicterus
Rickets
§
  • Neurological
Traumatic brain injury, such as the damage that is caused by Shaken Baby Syndrome
Lower Motor Neuron Lesions
Upper Motor Neuron Lesions
§
  • Miscellaneous  
Central nervous system dysfunction, including Cerebellar lesions
Hypothyroidism

Other names for hypotonia
  • Low Muscle Tone 
  • Benign Congenital Hypotonia 
  • Congenital Hypotonia 
  • Congenital Muscle Hypotonia 
  • Congenital Muscle Weakness 
  • Amyotonia Congenita 
  • Floppy Baby Syndrome 
  • Infantile Hypotonia