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Conquering a life of immobilityreturn to first page of SPS

"They didn't give him any antibiotics or even clean the wound," said Hollenbaugh's aunt, with whom he lives in Bandera.

For several months after the accident, he experienced post-concussion pain and possibly a bacterial infection that moved into his spinal column. Hollenbaugh soon noticed that the left side of his body began to immobilize. He couldn't pick his leg up to bend his knee and the nose of his shoes wore off before the sole showed signs of wear.

Hollenbaugh also experienced an increased sensitivity to loud noises. The sound of an ambulance or police siren sent shocks throughout his system and the left side of his body completely locked up.

Despite repeated and consistent complaints to doctors, he was told that his symptoms were imaginary. He was treated for pain management and nothing more.

According to information from the National Organization for Rare Disorders, doctors from Yale University have theorized that stiff-person syndrome may be an autoimmune disorder in which the body's natural defenses against invading organisms attack connections in the brain and spinal cord where gammaaminobutyric acid (GABA) flows between nerve ends, helping to modulate the central nervous system.

Leis explained that when the body has too little GABA or gammaaminobutyric acid, there is nothing shutting down the nervous system.



"When startled, your body freezes and becomes firm like a rock and you fall like a piece of lumber," Leis said.

Hollenbaugh has undergone a battery of treatments, including plasmapheresis, a procedure that separates blood cells from plasma to remove unwanted toxins and metabolic substances from the blood. The plasma is exchanged with other human blood and then retransfused back into the body. He also had between three and four inches of his spinal cord shaved off and replaced with a titanium capsule that feeds liquid medication to his body from a pump surgically implanted in his lower abdomen.

All of these measures are meant to treat and maintain his symptoms. There is no known cure.

Late last year, Hollenbaugh lost complete use of his legs. He needs a customized motorized wheelchair to fit his six-foot-four-inch frame because he does not have enough upper body strength or coordination to move a manual wheelchair.

He wears an eyepatch to eliminate the double vision that onset last Christmas and his speech has become slow and slurred.

Some days it's really hard to understand me and other days it's okay," he said. "It frustrates me. People treat me like I'm stupid because I sound like this, but I'm not."Because neither Medicare nor Medicaid will cover the costs of a disability van, he and his aunt have improvised with an elaborate homemade pulley system to get him in and out of her pick-up truck. But after the 10 minutes it takes just to get him seated, Hollenbaugh is left exhausted and out of breath.

 

What is Stiff-Person Syndrome?Stiff-person syndrome is a autoimmune condition with constant painful contractions and spasms of voluntary muscles, particularly the muscles of the back and upper legs. Symptoms may occur gradually, spreading from the back and legs to involve the arms and neck. Symptoms may worsen when the affected individual is anxious or exposed to sudden motion or noise. Affected muscles may become twisted and contracted, resulting in bone fractures in the most severe cases. Individuals with stiff-person syndrome may have difficulty making sudden movements and may have a stiff-legged, unsteady gait.  They tend to trip easily, fall and have seizure like spisodes.Sleep usually suppresses frequency of contractions. Stiffness may increase and patients may develop a hunched posture (kyphosis) or a swayback (lordosis).

Is there any treatment?  IVIG & stem cell replacement .

What causes SPS? Well, it's been known for over 20 years that most SPS patients have antibodies against the enzyme GAD65, which is required for the production of GABA, the main inhibitory neurotransmitter in the brain. The body shouldn't be producing antibodies against its own proteins, but unfortunately this does happen quite often, for various reasons, and the result is autoimmune diseases.

The drug IVIG,intravenous immunoglobulin may be beneficial. Physical and rehabilitation therapy may also be needed.  Plasmapheresis also helps. IVIg is a good first line treatment.Stem cells cure the condition. What is the prognosis?There are good chances of early remission if IVIg is used very early.. What research is being done?
Stem cell treatments and infections as the cause of SPS