The Man’s Blood Pressure Dropped, and He Was Acting Strange. What Was Going On?

https://www.nytimes.com/2019/06/19/magazine/low-blood-pressure-seizures-diagnosis.html

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“It’s my husband, isn’t it?” the woman asked the volunteer emergency medical worker directing traffic away from the restaurant where she was supposed to meet her spouse, who was then 71. The volunteer nodded grimly. She knew most of the emergency workers; her husband still volunteered with them. The wife hurried into the restaurant. When she made her way through the door, she saw him sprawled on the floor. His eyes were open but unfocused. His pants were soaked with urine.

“What took you so long?” her husband of nearly five decades mumbled irritably when she knelt beside him. What happened? she asked the emergency volunteer. Seizure, he answered quietly. Her husband had walked into the restaurant, greeted old friends and sat at a table. Then suddenly he slumped over, unconscious. Chairs and tables were moved aside, and the patrons helped him to the floor. His arms jerked for a minute or so, then stopped.

Moments later the volunteer medical team arrived. By the time his wife got there, the man was fully awake but confused. He didn’t want to get into the back of the ambulance once it arrived. “That’s for the sick people,” he insisted repeatedly. His wife, frustrated and scared by his odd behavior, shouted at him to just get in the ambulance. He allowed himself to be loaded into the vehicle. His wife followed as they drove to the hospital.

Blood Pressure Problems

This was the most recent in a series of medical encounters for this patient over the past six weeks. It started with his blood pressure. He’d had hypertension for decades, and it had been well controlled on a single pill for almost as long. But recently his blood pressure would swing from his usually high numbers to very low ones; it made him dizzy. Normal pressure for this patient was 130 to 140 over 80. But sometimes when he checked it now, the top number, the systolic pressure, was barely 100.

He stopped taking his blood-pressure medicine, and when after a week he felt no better, he made another appointment to see his internist, Steven Hersch. In the office, his blood pressure was in the normal range. But for a patient with high blood pressure who wasn’t taking his medication, that normal wasn’t normal. His exam was unremarkable, so Hersch sent him to the lab. His tests were fine: There was no anemia, no elevated white count, no kidney disease, no diabetes.

Feeling Unsteady

The patient felt worse and worse, and so a few weeks later, he, along with his wife, went back to Hersch. He felt off balance, he told the doctor. When he walked down the hallways at home, his wife added, he would put a hand on the wall, as if he was afraid he would fall. And sometimes his speech was slurred. Again, his exam was normal. But he did seem a little unsteady when he stood up from the chair. Hersch noted that the patient had been prescribed Ambien for sleep. Could that be an issue?

The doctor told him to stop taking the sleep aid and sent him back to the lab. Again, the tests were all normal, but the very next day Hersch got a call from the man’s wife, describing his collapse in the restaurant.

Seizures are not uncommon in the elderly. Nearly a quarter of those who have a first seizure are over 65. Most are caused by a stroke or a mass; traumatic head injuries can cause them. So can abnormalities in blood chemistry. In the emergency room, the man had no sign of any of these, despite a thorough exam and extensive testing and imaging.

Personality Changes

A couple of days later, the patient saw Hersch for a thorough physical exam. The doctor was worried by what he saw. As long as the doctor had known him, he smoked; he had high blood pressure, gout and arthritis; he was overweight and probably had sleep apnea, though he never found time to take the test. But despite all that, he always appeared an unstoppable force of nature. Now, though, he was forgetful and clumsy and was having these wild swings in blood pressure. He also seemed somehow different. While he had never been a quiet man — always chatty, energetic and eager to engage — these days, his wife reported, he never stopped talking. He was restless: constantly moving and rarely sleeping.

The patient finally agreed to get the sleep-apnea study. And an EEG was done — they would get the results in a few days. He was scheduled to see a neurologist the following week. It was worrisome, but everything seemed to be moving in the right direction.

A New Way of Thinking

But not fast enough. The day after he saw Hersch, the patient had a second seizure. This time, his wife heard a fall and ran in to see him jerking on the floor. Again he was rushed to the emergency room. When the man’s wife called Hersch as she drove to the hospital, the doctor started to wonder if he was approaching this patient from the wrong perspective. He was thinking of him as someone with new-onset seizures. What if the seizures were instead a result of whatever strange process was causing his sudden low blood pressure and his loss of balance? His logorrhea and restlessness?

What kind of disease could cause both neurological and psychiatric symptoms? he wondered. In reviewing his patient’s rapidly progressive course, and the extensive work-up that had already been done, Hersch knew that he had to move into thinking about unusual illnesses — zebra territory.

In his head he put together a list of the other possibilities: Multiple sclerosis could cause all kinds of strange neurological symptoms. Exposure to certain heavy metals could also result in this array of symptoms.

Memory of a Patient Past

Suddenly Hersch recalled another patient he saw years before. That patient also started having seizures, troubles with his blood pressure and changes in his personality. Tests ordered by that patient’s neurologist had allowed Hersch to diagnose an autoimmune encephalitis. These are a relatively new group of disorders in which the immune system wrongly attacks the brain. Treatment depends on strong immune-suppressing drugs, which can be lifesaving, although that patient died.

Hersch ordered tests for each of these disorders for his current patient. The results came in over the following week. It wasn’t M.S. It wasn’t any of the heavy metals. Then, two weeks after the man’s first seizure, the doctor got the answer he’d been looking for. It came from the Mayo Clinic in Rochester, Minn., one of the few labs able to test for immune-mediated diseases of the brain. He had an autoimmune encephalopathy; his symptoms were caused by an antibody that was first described in 2000, called Caspr2. The disorder caused by this rogue antibody is usually seen in men over 65 who, like this patient, develop fluctuations in blood pressure or heart rate, changes in personality, insomnia and problems with balance.

Saving Another Life

The patient was relieved to finally get a name for his strange collection of symptoms. He and his wife flew to the Mayo Clinic to start the treatment. His improvement has been slow, and even after three years, he isn’t fully recovered.

Several months after making this diagnosis, Hersch ran into the widow of the patient who died of autoimmune encephalitis. He told her that although he hadn’t been able to save her husband, what he learned from him helped save another life. It was news that, though hard to acknowledge, provided a little unexpected comfort to both doctor and widow.