Index
We are talking about the difficulty in controlling the muscles, which tend to stretch and weaken.
Hereditary spastic paraplegia, spastic paralysis, or Strumpell-Lorraine syndrome is a collection of inherited neurological disorders. There is the gradual weakening of the leg muscles and increased muscle tone and spasticity.
Spastic paralysis or Strumpell-Lorraine syndrome is also known as familial spastic paraplegia.
The age of onset of hereditary spastic paraplegia symptoms and the severity of weakness and spasticity is significantly variable.
Symptoms may be seen from infancy to as late as when a person is about 90 years old, but usually, signs of hereditary spastic paraplegia appear in their 30s.
What are the causes of hereditary spastic paraplegia?
Studies suggest that mutations in different genes cause inherited spastic paraplegia.
In most cases of inherited spastic paraplegia, these mutations tend to be transmitted in an autosomal dominant manner.
In sporadic cases, these mutations are transmitted in an autosomal recessive manner.
The exact defect in hereditary spastic paraplegia is still unknown. Still, it is suggested that some associated symptoms are caused by progressive degenerative changes in the corticospinal tracts, whose function is to transmit impulses from the brain to the muscles, which are responsible for the control of movements. Volunteers.
Subtypes of hereditary spastic paralysis
There are two subtypes of inherited spastic paraplegia. The two subtypes are the uncomplicated or pure type and the complicated type.
Uncomplicated or Pure Type: The uncomplicated or pure type of hereditary spastic paraplegia is associated with spastic paraplegia.
Complicated type: associated with spastic paraplegia and additional neurological abnormalities. Other neurological abnormalities are hearing impairment, mental retardation, and ataxia.
Simple or uncomplicated symptoms of hereditary spastic paraplegia
What are the symptoms of hereditary spastic paraplegia?
Symptoms of the uncomplicated or pure type of hereditary spastic paraplegia:
- Muscle and joint stiffness.
- Weakness: there is weakness in the leg muscles.
- Balance: involuntary falls are observed secondary to a balance disorder. The abnormal balance is caused by muscle weakness and malfunction of the cerebellum associated with hereditary spastic paraplegia / Strumpell-Lorraine Syndrome.
- Abnormal gait: The disease is associated with an irregular gait pattern, which progressively worsens as the disease progresses, but the total loss of the ability to walk is an infrequent phenomenon.
Additional symptoms suggest:
- Hearing impairment.
- Mental retardation.
- Ataxia.
How is it diagnosed?
To diagnose hereditary spastic paraplegia or Strumpell-Lorraine syndrome, it is essential to have information about the person’s family history to identify if any other family member or relative has the same problem.
Apart from this, other non-genetic causes of spasticity must be ruled out.
For this, spinal and brain MRI is imperative. An electrophysiological study is being investigated to assess the characteristics of the disease. 3 To confirm the diagnosis of hereditary spastic paraplegia, genetic tests are performed that target known genetic mutations.
Treatment
As of now, there is no precise treatment for hereditary spastic paraplegia or Strumpell-Lorraine syndrome, but some of the treatments mentioned are helpful.
Physical therapy is vital for improving the range of motion of the affected limb, as well as strengthening the muscles. A consultation with a specialist in physical medicine and rehabilitation can also be beneficial.
People with inherited spastic paraplegia have a high degree of spasticity and weakness in the leg muscles, making it difficult to walk.
For this, antispasmodics can be beneficial in reducing spasticity and helping to strengthen muscles with physical therapy.
Exercises for hereditary spastic paraplegia
Strengthening exercises: These exercises help strengthen muscles and prevent muscle atrophy development due to disuse.
Back pain resulting from inherited spastic paraplegia can be relieved with back strengthening exercises.
Stretching exercises: These exercises are beneficial in increasing the range of motion and preventing the development of cramps, etc.
Aerobic exercises: Apart from the activities mentioned above, regular aerobic exercises are also beneficial for people with inherited spastic paraplegia or Strumpell-Lorraine syndrome.
Some of the functional aerobic exercises are:
- To walk.
- Riding a bicycle.
- Water aerobics.
- To swim.
