My dad the medical mystery

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Wow, where to start.... I'm not sure what of this is relevant.

My father, who lives in the Bahamas, visited Israel(and Petra, Jordan) in October 2009. He was his normal, cranky, 69-year-old, Ashkenazi self. He has had a quintuple bypass, an aortic aneurysm repaired, diabetes, some skin malignancies removed, but otherwise was active and healthy. He got tired on some of the longer, hotter hikes., not surprising for a 69-year-old man.

In November, he complained that he feels like he never really "bounced back" from the Israel trip. He said he feels a bit blah, tired all the time, and that he doesn’t feel like doing anything. He'd become mildly forgetful, but not alarmingly so.

In the months after his return, he started to walk with more of a shuffle. By May 2010, he was walking like an old man and complaining that "it's no fun getting old." He had some trouble with his memory.

By mid June, he was noticeably forgetful, withdrawn, uncoordinated, and easily fatigued. He turned 70 on June 27, and he started to stumble and was getting confused. He was in Canada and had a CT scan that ruled out stroke. Coming out of the hospital, Dad complimented his wife on her fabulous parking job. He was the one who drove. He also asked her where she had slept the night before.

He flew home two days later. At the airport, he fell over a few times. He had trouble writing and forgot how to fill out a customs form, something he has done a hundred times before. At home, he sounded very tired and groggy, almost drunk. In one of his last moments of lucidity, while having trouble getting dressed one morning, he said, "I feel like a goddamn invalid."

The next morning, he was not well enough to get out of bed. Bloodwork and another CT scan didn’t indicate a problem, but basic cognitive tests indicated that something definitely wasn’t right. He had a hard time doing simple math problems and wasn’t sure where he was.

A mild case of case of conjunctivitis became a raging case while at the hospital. We flew him to the Florida hospital where he'd had the abdominal aortic aneurysm surgery in 2008. Shortly after admission there, Dad had tremors on the right side of his body, but those dissipated after a few days. From then, myoclonus could be provoked by bending his arms or ankles.

During a month in the hospital, he weakened to the point of becoming completely bedridden and incontinent. He slept most of the time and had fluctuating levels of cognition when he was awake. There were times he would be interested in what we were talking about, but he never really initiated discussion. There were days when he didn’t recognize his family. The cognition could fluctuate every ten minutes. For the most part, he’d stare at the TV blankly. When he was awake, he ate well, but he was sleeping so much that he needed a PEG tube.

He was put on acyclovir right away, but tests for these viruses were negative: West Nile, St. Louis, eastern equine, western equine, California, varicella, herpes, measles. Also negative for molds and bacteria. He had no fever.

The MRI showed "moderate degree of congestion and/or inflammation involving the right maxillary (sinus?)"

CSF 14-3-3 was high but tau was normal, the report said: "atypical for an acute encephalitis although there was possibility of a subacute or chronic encephalitis. On empiric doxicycline and acyclovir until more information becomes available."

PEP (serum) "consistent with acute inflammatory pattern"

Many different specialists tended to him, each with their own theories, but there was no consensus on the diagnosis. Tumor markers came back negative. EEG was slow, but not distinctive. He did have thyroid microsomal antibodies. Serum calcium was mildly high (12). Aphasia, ataxia, micrographia, etc. etc. - he had them.

By the process of exclusion, doctors suspected sCJD, Hashimoto’s encephalopathy, or paraneoplastic syndrome. They plugged him with an initial dose of 100 mg prednisone and discharged him, hoping for the best. The prednisone would taper to 60 mg and then to 40, etc.

He returned home and improved in August. He was awake every day for most of the day, clearly know who we all were, and did quite well with the family portrait game. His voice remained quiet and hard to understand, and he didn’t really initiate conversation, but he responded to questions without hesitation and maintained a sense of humor. But he was confused, talking about returning to his childhood home in Montreal and asking where his father (passed away in 1992) was and asking where the kids were. Physically, he was still weak and incontinent, but with a bit of work, we were able to get him to roll himself around in a wheelchair. He fed himself, albeit clumsily, and his appetite was quite good.

We all helped with exercises, with PT coming to the house a couple of times a week to do the heavy-duty work. With a bit of practice, we were able to get Dad to stand up in his walker all by himself.

On September 17, he choked on his breakfast. He's been on PEG feeding only since then. He started to sleep more and more. Near the end of the month, While I was away, his physician gave him 60 mg prednisone just to see what would happen. Within an hour, Dad was alert and kissed his wife when she asked for it, although there wasn’t a whole lot more than that in terms of communication. Over the next few days, on the same dosage of prednisone, his condition gradually reverted.

Since then, my father appeared to be in a vegetative state. He'd cough, sneeze, groan, open his eyes once in a while - but there was no indication of recognition. His hands and feet trembled and he'd roll his head. He responded to pain/discomfort anywhere on his body with groans and/or a wince. Another blood test showed still no tumor markers.

However, the physician again dosed him with a large amount of prednisone yesterday. Again he "woke up". He couldn't talk, but he was able to respond to questions with blinking. He puckered his lips to give his wife a kiss. He appeared to smirk and pant, which we interpreted as a laugh. He also cries.

So....

It's not Hashimoto or the initial steroids would have worked much better.

It's not CJD because the steroids wouldn't help at all.

If it's infectious, it's a disease that there's no known test for.

Any idea what it is? Any idea what to try next?

I have full medical records and MRIs for anyone who wants to take a look.