All in Your Head - Untreated Gluten Sensitivity
There is one Sunday morning that Mike Howell will never forget. It was about seven years ago, January 7, 2001. For Howell, the day began like any other Sunday. He woke early and dressed to attend worship service at the church across the street from the house he shares with Linda, his wife of 27 years. But this morning would be different. Howell walked into the church and lost his sight.
“Linda went on into the sanctuary but I wanted to check out the church bulletin board. As I approached it, I saw flashes of pink and blue lightning — and then I couldn’t see at all,” Howell says.
He stood in front of the bulletin board without moving, forcing his eyes open because he thought they were closed. Standing silently and alone, he broke into a cold sweat. The sensation in his eyes lasted two to three minutes, followed by a blasting ache in the back of his skull. Then his sight returned “like a small curtain rising.”
He proceeded quietly into the sanctuary, sat down next to his wife and whispered to her what had just occurred. After church, as the congregation filed out, Linda corralled a nurse friend and told her about Howell’s episode. At the nurse’s advice, the Howells drove to the local hospital where doctors ran a CAT scan, did some blood work and tests, suspecting a stroke. “But they couldn’t find anything,” Linda says. Howell was discharged with a headache and a follow-up referral with his primary care physician.
Mike Howell, 53, is a solid, consistent kind of guy, a hard-working man devoted to his wife and kids. Steady and reliable both as a family man and an employee, Howell, of Sodus, New York, worked as a stationary engineer for Eastman Kodak for 25 years, operating and maintaining industrial boilers. He rarely missed a day at the job and always showed up on time. Yet within weeks of the episode at church, Linda noticed that her steadfast husband was changing in alarming ways.
“He would have spells where he would forget how to get to work. He couldn’t spell his name,” Linda says. “It was very scary. I knew we were in trouble.”
Howell visited his primary care doctor, Jeannine Dolan, M.D., and then a neurologist. After examinations, more tests and an MRI, doctors found white matter lesions on his brain but no evidence of stroke.
“The neurologist wasn’t sure what was going on,” Linda says. By the end of January, the doctor was calling Howell “a mystery man.” He suggested a psychiatric exam and recommended a psychotherapy group.
In the meantime, Howell was suffering from violent headaches which didn’t respond to any of the usual over-the-counter remedies. “He was in so much pain that he would grab the back of his head and hold it,” Linda says. “Nothing we did would stop it.”
“The left side of my body was numb. I just didn’t feel right,” Mike recalls.
By the end of March, Linda could barely control her frustration and concern. The more the doctor talked about Howell’s “psychological problems,” the angrier she became. “I’d been married to this man for over two decades and I knew this wasn’t psychological,” she says. Sitting in the doctor’s office one day, her temper exploded.
“I totally lost it. I was so angry that I was shouting at the doctor and crying. He looked over at Mike and said, ‘It doesn’t look like your wife is handling this very well.”
They never went back.
A Long Journey Begins
A visit to a second neurologist provided no additional insight. By June, Linda took Howell to a third neurologist who conducted more tests, including a spinal tap, another MRI and an additional psychiatric exam to evaluate him for depression and memory loss. He was tested for Mad Cow Disease (Bovine Spongiform Encephalopathy, BSE), Multiple Sclerosis (MS) and other neurologic conditions.
Howell continued to suffer from debilitating headaches but now he had episodes of face droop and slurred speech.
“It was like he was drunk, like his mouth was full of novocaine,” Linda says.
Howell would have periods where he didn’t recognize Linda or their children. “There was no rhyme or reason to it. He would be completely out to lunch, have no idea where he was or who I was … and then it would go away,” Linda says.
In early December, Howell was diagnosed with Binswanger Disease, a rare and progressive form of dementia that is characterized, among other things, by memory and cognition losses and difficulty in walking and speaking. There is no cure. For the Howells, it was a devastating death sentence.
“Mike was given five years to live,” Linda says.
Already grieving the loss of the strong husband she loved and now faced with his inevitable slow decline with no hope for recovery, Linda mourned deeply. To honor their life together and celebrate the vibrant years they had had, as well as the limited time still remaining, the Howells decided to renew their wedding vows. They planned a summer ceremony and invited friends and their extended family.
“The family was all there. They came in from all over. Our sons and grandson walked me down the aisle,” Linda says. “It was wonderful.”
A New Answer
As the months progressed, Howell continued visiting doctors for various tests and ongoing treatments. Additional MRIs revealed more white matter lesions in his brain. He underwent chelation therapy to address heavy metals in his blood due to working in an industrial environment. As his symptoms worsened, he became increasingly dependent and began using a wheelchair. Settling down for the long haul, Linda quit her job in order to take care of him full time.
In October 2004, Howell was referred to yet another doctor for a consult — Heidi Schwarz, M.D., a neurologist at the University of Rochester Medical Center and assistant professor of neurology at Strong Memorial Hospital. Almost immediately, the Howells sensed this doctor was different. Schwarz examined Howell, reviewed his medical history and listened intently to Linda describing her husband’s symptoms. “She never once looked at us like we were crazy,” Linda says.
“I was the fourth or fifth neurologist that Mike had seen. He had an abnormal MRI scan and risk factors for stroke,” Schwarz says. “We were trying to control those risk factors … and yet he was getting worse.”
During one subsequent visit while Schwarz was examining Howell yet again and “we were scratching our heads,” Schwarz says, “Mike’s wife happened to mention that some lab work years back had showed that Mike had a gluten problem. I said, ‘It showed what? Well, I’ll be darned!”
For confirmation, Schwarz repeated the blood test. Results revealed that Howell did, in fact, have sensitivity to gluten. With that, in January 2005, she nailed his diagnosis: gluten ataxia.
Gluten ataxia, a disorder of the immune system, belongs to the same spectrum of gluten sensitivity as celiac disease and dermatitis herpetiformis. In these conditions, a heightened sensitivity to gluten, the protein in wheat, barley and rye, creates an increased level of allergy-fighting antibodies (specifically, antigliadin IgG and IgA) that turn on the body and attack it.
Symptoms of gluten sensitivity can be numerous and vary widely from individual to individual. Generally speaking, celiac disease is evidenced in the gut by damage to the small bowel. Dermatitis herpetiformis is evidenced on the skin by an itchy rash. With gluten ataxia, the focus of disease activity is in the brain, specifically the cerebellum, the center that controls coordination and complex movements like walking, speaking and swallowing. Often, the peripheral nerves located outside the spinal cord are also involved, leading to chronic and progressive neuropathy, a disease affecting the nervous system that results in feelings of numbness, tingling or pain.
Ataxia means clumsiness or loss of coordination. Symptoms of gluten sensitivity with neurologic manifest-ations are slurred speech, loss of coordination in upper and lower limbs, difficulty with normal walking, ocular problems, chronic headaches. It may affect the fingers and hands, the arms or legs, the body, speech or eye movements. In children and young adults, gluten ataxia can also cause developmental delay, diminished muscle tone, learning disorders and ADHD.
Recent studies indicate that gluten ataxia is a common cause of sporadic idiopathic (of unknown origin) ataxia, accounting for up to 40 percent of cases. Yet despite its prevalence, the disease isn’t well known and diagnosis is frequently missed. The reason? Doctors often look for gastrointestinal distress before they will consider the possibility of gluten sensitivity.
“Gluten ataxia is out there but so few of us have seen it — or perhaps recognized it. If you see a patient who has malabsorption problems, they can’t tolerate this or can’t tolerate that, if they have gastrointestinal complaints along with neurologic symptoms, then you order the antibody tests,” Schwartz says. “Yet most patients I see with neurologic manifestations of gluten intolerance don’t have a lot of GI symptoms, if any.”
According to a 2003 study published in Brain, gastrointestinal symptoms are present in only 13 percent of patients with gluten ataxia. “It’s been estimated that for every one patient with celiac disease who presents with GI complaints, there are seven patients with celiac disease who have no GI symptoms …. Only a proportion of patients presenting with neurological dysfunction association with gluten sensitivity will also have GI symptoms.”
The bottom line is that gluten sensitivity can be primarily — and at times exclusively — a neurologic disease.
“This is a disease that’s difficult to diagnose unless you maintain a low threshold of suspicion,” Schwarz says.
The blood panel to screen for gluten ataxia is the same used to ascertain gluten sensitivity. It measures the antigliadin antibodies (IgG and IgA) circulating in the blood, along with the endomysium and tissue transglutaminase antibodies. “A small bowel biopsy is not needed if symptoms are neurologic and antibodies are positive,” Schwarz says.
“If antibody results come back positive, I’ll send the patient to a gastroenterologist to do a small bowel biopsy to check for classic changes attributable to celiac disease, but even if this is normal, I will still recommend a six-month trial on a gluten-free diet,” Schwarz says.
Research by Dr. Marios Hadjivassiliou and colleagues published in Brain in 2003 states, “.... IgG antigliadin antibodies by definition remain the best diagnostic marker for gluten ataxia.”
Diet for Life
It used to be that Mike Howell’s breakfast of choice was a bowl of shredded wheat. For lunch, he’d grab a sandwich, sub or pizza. But these days, Howell stays away from his old favorites. The treatment for gluten ataxia is a strict gluten-free diet.
“I love donuts, muffins and bagels and I still would like to be able to eat this stuff,” Howell says. “But I know that if I touch any of it, I’ll end up more brain damaged.”
Linda has enthusiastically embraced all aspects of gluten-free eating, creating gluten-free pumpkin pie, bagels, and pasta for her husband. “When he gets even a quarter gram of gluten, he’ll have a problem. In less than 24 hours, there’s a massive change. He’ll look at you as you talk to him and he won’t understand what you’re saying. His legs will drag, he’ll have unsteadiness, and slurred speech,” Linda says.
“Then it takes me at least two to three days to get back to where I was,” Howell says.
After many months on the gluten-free diet, Howell has shown remarkable improvement. His progress has been slow but steady.
“Remember that this is an auto-immune disease where antibodies attack other cells in the body. Once the immune process starts, it sort of has a life of its own,” Schwarz says. “That’s why going on a gluten-free diet can take months to see an effect with these patients.”
Today, Howell is out of the wheelchair and walking on his own. His thinking has cleared. And the violent headaches he used to endure on a daily basis? “I haven’t had one since April,” he says.
Howell’s sensitivity to even trace amounts of gluten remains very high. Schwartz says this type of reaction isn’t unusual.
“If a patient’s antibody levels are up and, for example, they prepare their food on the same countertop surface as everyone else, their symptoms will return. That’s even when they’re careful not to eat anything with gluten in it,” Schwarz says. “Gluten is in a surprising number of products, like toothpaste and medicines. Whenever I put anyone with gluten sensitivity on a medication, we check with the manufacturer to make sure it’s clear.”
Schwarz’s advice for patients who have episodic neurologic symptoms with no apparent known cause? Be persistent with your medical team.
“Almost every patient I’ve seen with gluten sensitivity has been dismissed somewhere along the line with a psychological diagnosis like depression or anxiety. If a person is suspicious that they may have gluten sensitivity, they should approach their doctor with the idea that this disease is very difficult to diagnose and has a broad array of clinical manifestations,” Schwartz says. “A lot of doctors had seen Mike Howell and had said they just didn’t know. That’s what’s going to happen to these patients unless they’re persistent. You have to be comfortable in advocating for yourself. It’s the squeaky wheel that gets the oil.”