Introduction:
Paraparesis is the partial inability to move the legs. Weakness of the hip and legs can also be seen in paraparesis. Paraplegia is different from paraparesis, and paraplegia usually refers to severe weakness and total inability to move legs. Spastic paraparesis is a group of disorders that has a common feature of weakness and stiffness of the muscles of the leg that is progressive.
What Is Spastic Paraparesis?
Spastic paraparesis is a group of rare neurologic disorders that cause gradual weakness accompanied by abnormal tightening of the leg muscles. Weakness of the muscles becomes worse over time. Spastic paraparesis can begin at any age and may affect both males and females equally. The hereditary form of spastic paraparesis was first described in 1883 by Strumpell, and later on, Lorrain added more details regarding the disorder. Hence spastic paraparesis is often known as Strumpell-Lorrain syndrome.
What Causes Spastic Paraparesis?
The causes of spastic paraparesis are as follows:
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Hereditary Spastic Paraparesis:
The hereditary form of spastic paraparesis is caused due to abnormal genes and it may be passed on from parents to children. The faulty genes lead to the progressive damage of the spinal nerves which control movement and muscle tone of the lower body. Damage to these nerves results in gradual disability in leg movements. Even though most of the children with a family history of hereditary spastic paraparesis may be a carrier of the abnormal gene, all the children in the family do not develop this disease. In few cases, spastic paraparesis develop randomly due to genetic change without any family history of the disease.
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Tropical Spastic Paraparesis:
Tropical spastic paraparesis is caused by human T-lymphotropic virus type 1 (HTLV-1). In tropical spastic paraparesis, the spinal cord is affected followed by immune system weakening that leads to muscle weakness and loss of sensation in the legs. The HTLV-1 virus can be transmitted from person to person through the following ways:
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Unprotected sexual activity.
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Breastfeeding.
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Sharing infected needles among drug users.
What Are the Symptoms of Spastic Paraparesis?
The symptoms of spastic paraparesis are as follows:
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Gradual stiffening and weakening of the legs.
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Difficulty in walking that leads to an increased risk of falling.
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Fatigue.
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Pain in the hands, feet, ankles, hip, and knee.
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Loss of balance.
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Difficulty in learning and remembering new things.
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Pricking or burning sensation in the hands and feet.
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Bowel and urinary dysfunction (experiencing the need to pee even when the bladder is not full).
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Hearing or vision problems.
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Absence of sensation in the feet.
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Lack of coordination.
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Ichthyosis (Scaly, dry, and thick skin over large areas of the body).
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Seizures (uncontrolled sudden disturbances in the brain).
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Back pain radiating to the legs.
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Walking on the tip of the toes with the feet turned inwards.
How to Diagnose Spastic Paraparesis?
The methods used to diagnose spastic paraparesis are as follows:
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Medical History and Physical Examination:
The doctor collects a detailed history of the patient regarding the symptoms like difficulty in walking and pain in the legs. In case of hereditary paraparesis, the doctor enquires about the family history to find out the presence of paraparesis among the family members. In the case of tropical spastic paraparesis, the doctor finds out the chances of exposure to HTLV-1 virus by asking the patient about sexual history and drug injections. The doctor also rules out the possibility of other diseases which cause similar symptoms.
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Magnetic Resonance Imaging (MRI):
MRI is an imaging test that uses computer-generated radio waves and magnets to produce detailed images of the body's organs. MRI of the brain and spinal cord is useful in the diagnosis of spastic paraparesis as it helps to rule out the possibility of other disorders and also to find out the abnormalities of the brain and spinal cord. Specialized MRI techniques like diffusion tensor imaging help to detect nerve abnormalities in spastic paraparesis cases.
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Genetic Testing:
Genetic testing is performed to find out the defective genes in patients with hereditary spastic paraparesis who have a family history of the disease. Genetic testing is often used to confirm the diagnosis of hereditary spastic paraparesis.
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Blood Tests and Lumbar Puncture:
Blood tests and lumbar puncture (a test which is done by inserting a needle in the lower back region to obtain the cerebrospinal fluid present in the spinal cord) are useful in cases of tropical spastic paraparesis to find out the presence of HTLV-1 virus. Blood tests done to find out the number of cells present in the blood and serum electrolytes are useful to exclude the possibility of other disorders.
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Electromyography (EMG) and Nerve Conduction Studies:
Electromyography is used to measure the electrical activity of the muscle in response to nerve stimulation. Nerve conduction studies evaluate the speed of electrical signals that travel through the nerves, which makes the muscle react. Both these tests can be used to find out the disorders associated with muscle or nerve dysfunction and problems in signal transmission between nerves and muscles.
How to Treat Spastic Paraparesis?
The treatment measures of spastic paraparesis aim at relieving the symptoms experienced by the patients. The various methods used are as follows:
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Medications:
Muscle-relaxing medications help in reducing the weakness and stiffness of the muscles, thereby improving the ability of the patient to walk. Medications to control bladder problems and reduce urinary urgency are given.
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Exercise:
A regimen of regular physical exercise under the guidance of a physical therapist is recommended. The exercise programs often begin with low intensity and are formulated under the combined supervision of a neurologist and physical therapist. Exercise helps in maintaining cardiovascular fitness, reducing weakness and stiffness of the muscles, and improving the ability to walk thereby reducing the risk of falling.
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Occupational Therapy:
Occupational therapy helps the patient to get adapted to the disability both mentally and physically. This therapy helps the patient to carry out the daily activities of life without any difficulties under the guidance of an occupational therapist.
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Orthotics:
It is a custom-made shoe insert or ankle brace that is used in treating problems of the feet and ankle. In patients with spastic paraparesis, ankle-foot orthotics help to reduce the tendency of foot extension that causes toe dragging. Often ankle-foot orthotics are used in combination with medicines that reduce muscle stiffness.
Conclusion:
Spastic paraparesis is a serious condition that may become worse over time. Various studies regarding gene modification and gene transfer to treat spastic paraparesis are under research. Since these advanced treatment measures are under research, patients are often treated with the help of medications and a regular exercise routine. Hence early diagnosis and prompt treatment help the patient to get relief from the symptoms and lead a normal life.
