The kudo in Saturday’s Bee read, “To Dr. Wheeler, Rob, Lori and BGH emergency room staff. Thank you for the prompt, caring, gentle and professional care you gave our daughter treating the pain she was experiencing as a result of a seizure brought on by Stiff Person’s Syndrome.”

Also called Moersch-Woltman Syndrome for the physicians who coined the term Stiff-Man’s Syndrome in 1956, neither of these terms defines this rare disease that affects twice as many women as men and can affect children as well as adults. The common name today, Stiff Person’s Syndrome, according to Medscape.com should really be Stiff Individual Syndrome, but that sounds a bit like they’re nit-picking.

The name isn’t important. The disease affects one in a million people and of those affected only 10 percent is known to have seizures, so we have one among us who is a truly unique patient, and a medical staff that truly deserves accolades.

Johns Hopkins Medicine’s website gives us a description: “Stiff Person Syndrome (SPS) is a rare disease of the nervous system. Progressively severe muscle stiffness typically develops in the spine and lower extremities. Most patients experience painful episodic muscle spasms.

“An auto-immune component is typical and patients often have other auto-immune disorders. Symptoms usually begin in the mid-forties. The severe muscle stiffness of SPS responds to benzodiazepines.”

Benzodiazepines, you may know them as Valium, Librium or Xanax, act as a central nervous system depressant. People also take these drugs for anxiety, insomnia and they are often used for managing back pain.

Because the disease is so uncommon, most of the explanations I found on the internet were in medical lingo that made my eyes cross. But, I’ll try to interpret what I read as accurately as I can. If you suspect that you or someone you know has this disease, please trust your healthcare provider, not me!

Stiff Persons Syndrome starts gradually in the axial muscles (skeletal muscles of the trunk or head) which may confound your medico. Med-scape.com says, “In the initial stages of the disease, the patient has an exaggerated upright posture and may report back discomfort or stiffness or pain in the entire back, which is worse with tension or stress.”

Sometimes the symptoms occur briefly and resolve themselves either within hours or days making diagnosis difficult. Often doctors will think the symptoms are psychological rather than physical.

As it progresses the proximal (nearer to the trunk of the body) limb muscles become affected particularly when the patient is stimulated. Surprise, fear, anger and loud startling sounds can result in painful severe spasms of the arms and leg muscles. It’s no surprise that the patient may begin to suffer from depression.

As the disease progresses, few muscles are spared and joint deformities may occur. Spasms may result in bone fractures or muscle ruptures. At this stage, performing daily activities can become problematic.

Besides the drugs mentioned above, there are other treatments. Johns Hopkins uses a multi-modality approach with therapies that include intravenous immune-globulin injection (just what it sounds like, IG is a medication), plasma transfusions, physical and behavioral therapy.

This is probably more than you wanted to know about the disease, but the point is that we have an extremely well-educated medical staff at Bonner General Hospital and we should all be proud although not surprised that this patient was treated efficiently, effectively and courteously.

Kathy Hubbard is a trustee on Bonner General Hospital Foundation Board. She can be reached at 264-4029 or [email protected].