Overview
Diagnosis
There is no single test that can confirm a diagnosis of primary lateral sclerosis. The condition shares symptoms with other neurological disorders, including multiple sclerosis and amyotrophic lateral sclerosis. Because of this overlap, diagnosis usually involves multiple tests to rule out other causes.
A healthcare professional begins by reviewing your medical and family history and performing a detailed neurological examination. Additional tests may then be recommended.
Blood tests
Blood tests are used to look for infections or other medical conditions that could explain muscle weakness or neurological symptoms.
MRI scans
MRI or other imaging studies of the brain and spinal cord may show signs of nerve cell damage. These scans can also help identify other possible causes of symptoms, such as structural abnormalities, multiple sclerosis or spinal cord tumors.
Electromyogram
An electromyogram measures the electrical activity of muscles both at rest and during contraction. The test helps evaluate whether lower motor neurons are involved. This distinction is important because primary lateral sclerosis affects upper motor neurons, while ALS involves both upper and lower motor neurons.
During the test, a small needle electrode is inserted through the skin into selected muscles.
Nerve conduction studies
These studies measure how well electrical signals travel through your nerves to the muscles. A low electrical current is used to detect nerve damage in different areas of the body.
Lumbar puncture
A lumbar puncture, sometimes called a spinal tap, may be done to rule out multiple sclerosis, infections and other conditions. A thin needle is inserted into the spinal canal to collect cerebrospinal fluid, which surrounds the brain and spinal cord. The fluid is then analyzed in a laboratory.
In some cases, it can take three to four years to confirm a diagnosis. This delay occurs because early ALS can resemble primary lateral sclerosis until additional symptoms develop. Repeat electromyograms over several years may be needed before the diagnosis is confirmed.
Genetic testing may be considered when juvenile primary lateral sclerosis is suspected. Genetic counseling may also be recommended in these situations.
Treatment
There are currently no treatments that can prevent, stop or reverse primary lateral sclerosis. Treatment focuses on easing symptoms, maintaining independence and preserving quality of life.
Medication
Medicines may be prescribed to manage symptoms such as:
-
Muscle spasms, also known as spasticity. These may be treated with oral medications such as baclofen, tizanidine or clonazepam.
-
Severe spasticity that does not respond to pills may be treated with a surgically implanted pump that delivers baclofen directly into spinal fluid.
-
Emotional changes, including sudden or intense emotional shifts, which may improve with antidepressant medication.
-
Drooling, which may be reduced with medicines such as amitriptyline.
Physical and occupational therapy
Stretching and strengthening exercises help maintain muscle strength, flexibility and range of motion. Therapy can also help reduce the risk of joint stiffness and loss of mobility and support daily activities as symptoms progress.
Speech and language therapy
Speech therapy can be helpful if facial or throat muscles are affected. Therapy may improve communication and assist with swallowing difficulties.
Nutrition support
If chewing or swallowing becomes difficult, a dietitian can provide dietary advice, nutritional supplements or specialized feeding strategies. These approaches help maintain adequate nutrition and body weight.
Assistive devices
As symptoms worsen, assistive devices may become necessary. Physical and occupational therapists can assess needs over time and recommend braces, canes, walkers or wheelchairs. Assistive communication devices may also help support speech and interaction as the disease progresses.
Advertisement
