Calling the house neurologist

Remember last year, when it took a while to diagnose what disease I had contracted? This year, it’s my dad’s turn – and it’s a hell of a lot more serious.

Let me start with his current condition. We thought he was in a classic vegetative state. He could move a bit, moan, open his eyes, react to pain, but show absolutely no cognition at all. He has a PEG tube for feeding but no other devices.

Three days ago, he received 60 mg of prednisone (a corticosteroid). The next day, he opened his eyes and could communicate by blinking and moaning, all appropriately to the questions asked. At one question my brother asked, my dad answered, “Sure”. That was probably instinctual, but it was the first word he’s used in months.

How did he get here? That I can tell you. What is causing this? That’s a mystery. If you have any idea, or know someone who might have any idea, please get back to us. We can supply all medical records as well as MRIs.

Here’s what happened….

Following is a synopsis of the progression of my father’s condition. Pre-existing conditions are a history of atrial fibrillation (on Coumadin), a quintuple bypass in 1993, a AAA (abdominal aortic aneurysm) in 2008, hypertension, adult-onset diabetes, benign prostate hypertrophy, dyslipidemia, hypertriglyceridemia.

October 2009
Dad, his wife Marion, and my brother visit Israel for ten days. Dad seems normal but got tired on some longer, hotter hikes – not surprising for a 69-year-old man. Around this time, he has an erythmatous, non-raised, macular rash on his right hip and lower back for four weeks.

November 2009
Back home in the Bahamas, Dad feels like he never really recovered or “bounced back” from the Israel trip. He says he feels a bit “blah”, tired all the time, and that he doesn’t feel like doing anything. Marion also notices that Dad has become mildly forgetful, but not alarmingly so – about what you’d expect from an older man.

May 2010
Dad shuffles and stoops over a bit when he walks, like an old man. No one is alarmed.

At a high-school-reunion softball game in Montreal, Dad is surprised at how weak and slow his body is. He remarks that he could barely throw the ball, and he fell down at least once. It’s been a while since he’s played, but he doesn’t think he should have lost as much ability as he did. Dad complains that “it’s no fun getting old.”

Early June 2010
Dad flies his Cessna over to Florida, a short trip he does all the time. On the way back, a friend flies the plane while Dad operates the radio. The friend notices that Dad is forgetting how to use the radio properly and is unacceptably slow in his communication. He takes over radio duties from my Dad.

June 17, 2010
On the way to Montreal, Dad acts confused and almost leaves his passport behind on the seat at the gate. It’s unlike him. He speaks with little volume, almost a whisper. When asked to speak up, he doesn’t, really. Overall, he seems tired and quiet. While throwing a football around with his sons, Dad seems uncoordinated. One son says, “Something’s not right with him.”

June 19-20, 2010
At his grand-daughter’s bat mitzvah, Dad sits unusually quietly. He can’t even be bothered to leave a table when people he has little regard for sit down there. Again, that’s not like him.

June 27, 2010
Dad turns 70 and we celebrate with a dinner. He seems really quiet, and confused. He asks when Child Three has hockey when he should know that Child Three as doing only baseball this summer. It’s a steep decline in the matter of a week. We suspect a stroke.

June 28, 2010
Dad has a CT scan, which comes back normal – no stroke. Upon leaving, Dad compliments Marion on her fabulous parking job, although he had driven to the hospital. He asks Marion where she’d slept the night before, which was with him. Dad struggles with driving, going too fast and making a few wrong turns.

June 29, 2010
At the airport, Dad falls over while going through security and again when taking his seat in the plane. He has trouble writing and forgets how to fill out a customs form, something he’s done a hundred times.

End of June, 2010
At home in Freeport, Dad feels tired and “not right.” On the phone, and he sounds groggy, almost drunk. His IM conversation is almost pure gibberish.

July 3, 2010
Dad has trouble getting dressed. Marion helps him, and he says, “I feel like a goddamn invalid.”

July 4, 2010
The next morning, Dad is not well enough to get out of bed. His local physician administers a mini mental state (MMS) exam. Dad is disoriented to time and place and has trouble with basic math. His handwriting is legible but small. A second CT scan and a blood test reveal no abnormalities. A mild case of case of conjunctivitis becomes severe while at the hospital. Since Dad’s condition seems to be deteriorating, he flies by air ambulance to JFK Medical Center in West Palm Beach, Fla.

July 2010
Shortly after being admitted, Dad has tremors on the right side of his body, but those dissipated after a few days. Afterward, myoclonus could be provoked by bending his arms or ankles.

Over the month, Dad weakens to the point of becoming completely bedridden. He sleeps most of the time and has fluctuating levels of cognition when he was awake. The fluctuations vary by day and even by hour. Sometimes all he does is stare at the TV. Sometimes, he’s talkative but in a completely different time and place. Sometimes, he’s all there. Sometimes, he doesn’t recognize anybody. We try to get him to name people in a recent family portrait. On his best days, he gets two or three names. We play cards with him, asking him to add the card values. He can and says it’s boring, but he can’t play blackjack.

When awake, Dad eats well, but he sleeps so much that he gets a PEG feeding tube.

JFK runs every test they have. Many different doctors and specialists tended to him but there’s no consensus. Abnormal test and exam results are:

Glasgow: 12/15 (best verbally disoriented)
MMS: 7/30
Strength: 4/5 upper, 2/5 lower
CBC: WBC 8.7 (70.4% neutrophils, 14.3% lymphocytes, 14.3% monocytes)
CMP: calcium10.2, albumin 3.8, BUN 26
Urine: specific gravity 1.023 (a shade high), protein 100 (2+), occult bacteria
Brain MRI w/o contrast: mild degree of senescence
EEG: slow waveforms; intermittent bursts of slowing at about 2-3 seconds, 8 Hz
alpha very transiently
CSF: protein 52.2 (mildly elevated), few WBC, possible subacute/chronic encephalitis
14-3-3: elevated (but total tau is normal)
Thyroid microsomal antibodies: 101.0 (high)
PEP serum: albumin 3.1 (mildly low), globulin 4.0 (high), alpha 2 1.2 (high), beta 1.3 (high), all consistent with acute inflammation
PTH Intact: 60.1 on July 23 (high normal), 109.5 on July 25
Ionized calcium: 1.55 (high)
Corrected calcium: 11.4 on July 23 (high), 10.7 on July 25 (high)
Vitamin D25: 15.9 (low)
Phosphate: 2.1 (low)

Note that his past medical records also indicate high levels of calcium. He was put on empiric doxicycline and acyclovir.

Tests are negative for culture growth, cryptococcal antigen, West Nile, St. Louis, Eastern Equine, Western Equine, California, influenza, Epstein-Barr, syphilis, herpes, HIV, RPR, mycoplasma, varicella, measles, brucella, Lyme, lead, mercury. Tumor marker tests are all negative.

By the process of exclusion, doctors suspected Creutzfeldt-Jakob disease, Hashimoto’s encephalopathy, or paraneoplastic syndrome. Whatever it is, it’s atypical.

Hashimoto’s is an auto-immune disorder that steroids can control. Dad gets 100 mg prednisone to start. Even before the steroid course starts, Dad starts to show some consistent improvement. He can stand with support and he’s a bit more lucid than he has been. With a prescription for steroids, Dad is discharged and he flies to Freeport to check into the hospital there. In Freeport, he spends most of the time sleeping.

July-August 2010
The steroids do not cure Dad, so Hashimoto’s is ruled out. Given that whatever Dad has is probably not treatable, we set up a hospital bed at his home and hire a caretaker and a physiotherapist to help Marion look after Dad’s basic needs.

Over the next couple of weeks, we do our own research. Among the conditions we suspect are Lewy body dementia and hyperparathyroidism. Nothing matches exactly. Together, we put together a spreadsheet with symptoms and conditions.

Dad is doing better than he was in July, however. His prednisone is down to 60 mg/day. He’s awake every day for most of the day. His voice remains quiet and hard to understand, and he doesn’t initiate conversation, but he responded to questions without hesitation and maintains a sense of humor. He knows who we all were, and does quite well with the family portrait game. We can engage him with some very basic games on the computer.

But he’s still confused. He talks about returning to his childhood home in Montreal and asking for his father (who passed away in 1992) and the kids. He is still weak but we can get him to roll himself around in a wheelchair. He feeds himself clumsily, but his appetite is good. With practice, Dad can stand up in his walker all by himself.

September 14, 2010
Marion takes Dad out for a ride in the car. Dad cries. Marion asks if he’s crying because he’s happy or sad and Dad says he’s happy.

September 16, 2010
Marion takes Dad for another drive. Dad asks Marion if she has the passports, because he thinks they’re leaving for Montreal. When Dad gets out of the car, he thanks the caretaker for everything, and kisses Marion goodbye and tells her that he’d see her in Montreal.

September 17, 2010
Dad chokes on breakfast, so we begin feeding only with the PEG tube. Dad has been mostly sleeping since.

October 2010
Dad has deteriorated. He is sleeping almost all day. His fingers are clenched, and his palms are turned down. His hands tremble slightly, and his tongue darts out of his mouth, as if he is playing with his mustache. When turned on his back, his head lashes violently from side to side.

Physical activity is limited to passive range of motion exercises when he sleeps. He opens his eyes occasionally, with no sign of recognition. He is completely non-verbal, his only communication being groans when we move him in bed. He sounds remarkably normal when he hiccups and yawns, but coughs are a bit of an effort.

October 12, 2010
The doctor gives Dad a booster of 60 mg prednisone. Within an hour, Dad is alert and kisses Marion when she asks for it. There’s no more than that in terms of communication. Over the next few days, on the same dosage of prednisone, Dad’s condition gradually returns to its former state. The theory is that the prednisone temporarily reduced brain swelling, which allowed it to function more normally.

After this, Dad appears for all intents and purposes to be in a vegetative state.

November 2010
Dad seems to have stabilized. His breathing isn’t as labored and and his head rolls back and forth a bit, but it’s not lashing violently anymore. His hands and feet still tremble. His eyes seem to open a bit more frequently. It seems like he might be tracking motion and making eye contact, but I can’t say that there is real recognition. He responds to pain/discomfort with groans.

November 10, 2010
The doctor puts dad on 60 mg of prednisone per day. On the second day, Dad opens his eyes for most of the day and shows alertness. He is able to answer simple questions by blinking his eyes and even appeared to laugh at jokes. He also cries a few times.

He is currently still in this phase.

We, and every MD we speak to, remain stumped. His fluctuating cognition seems to rule out CJD. The inconsistent response to steroids rules out Hashimoto’s. A blood test at the end of October still proved negative for all tumor markers, so it’s not likely paraneoplastic. Medication that brought the calcium levels to normal did not lead to any improvement, so it’s not likely hyperparathyroidism. It’s too quick a decline for Lewy body dementia, and really too quick for Alzheimer’s. Someone recently suggested amyotrophic lateral sclerosis (ALS).

Unless someone out there has any brilliant solutions, the only way we’ll know for sure is with an autopsy.

9 thoughts on “Calling the house neurologist

  1. I’m so sad to hear about your father 🙁 My grandmother is experiencing more and more signs of ageing, but thankfully, it seems to be all part of the natural process so far.

    From watching a lot of House, the first thing that comes to mind is a parasite or something of that sort. But clearly, I have no solid medical background to base that on.

    I’ll pass this on to my friends though! I hope you guys figure it out soon!

  2. Hi,

    The little experience I have is steering me towards a form of encephalitis, and despite the process of elimination, the positive results obtained with the use of corticosteroids is indicative of an inflammatory/autoimmune component. Now that being said, I’d love to see the CSF data updated. If he’d been septic from a bacterial infection, he would not have made it this far; the positive CSF indicates a breaching of spinal space integrity but could also be longstanding. The most puzziling aspect here is the fact that this condition was a sudden onset but steady progression; no infection, parasitic or viral or bacterial, can do that since these disease rely on proliferation of the microorganisms involved. With support, any toxin would have been past by now, and his LFTs would have been elevated during that time because of the liver’s increased activity.
    Viral cn never be eliminated because of the atypical response of the body; I would strongly suggest writing John Hopkins and UCLA about your father, as top reasearchers would be able to work as a team with the latest data to assist you.
    Therapeutically, you may ask to try using dexamethasone(Decadron) to isolate the part of the body involded. Also in the family of corticosteroids, Decadron targets intercranial pressure and spinal pressure. If this is a CNS issue, you’ll see improvement. If not, consider generalized demyelenating possibilities or look to viral. Something has to have triggered the onset of this.

    In the meantime, hang in there, I hope your father has some good days still and that you never lose hope in him regaining some quality of life. I admire your perseverance and love for your family.

    All the best,

    Christine N, RN

  3. I am so sorry for you and every person going through this with a loved one – including me with my husband who does have Lewy Body Dementia. Although only diagnosed recently after non-diagnoses and mis-diagnoses (Vascular Dementia, diabetic neuropathy), he has likely had it for at least four-five years, perhaps longer. This may be the case with your father, too, so the decline may seem too quick, but it can escalate very rapidly.

    Have you looked at for information on LBD? There is a lot of information there.

    These conditions are so insidious and affect the entire family. I hope that you can determine what it is so that at least some of the symptoms can be treated. You and your family are doing what you can by being your father’s advocate and getting as much information as you can.

    Do you have a list of all of the meds he has been given? People with LBD react negatively to quite a few meds. Is one of your doctors a geriatrician or a geriatric neurologist who has experience with LBD?

  4. Here’s the drug list:

    Amlodipine besylate 10 mg/day
    Lisinopril 20 mg/day
    Metoprolol tartrate 100 mg twice/day
    Metformin 500 mg three times/day
    Insulin on a sliding scale
    Simvastin 10 mg/day
    Fenofibrate 145 mg/day
    Tamsulosin 0.4 mg/day
    Dutasteride 0.5 mg/day
    Warfarin 4 mg/day
    Omeprazole 40 mg/day
    Allopurinol 300 mg/day

  5. His workup seems thorough and they’ve probably thought about this but felt a need to mention anyway – any evaluation for hydrocephalus? I thought of that because the classic presentation includes progressive dementia and gait disturbance, which sounds like his earliest symptoms. The third common early symptom is incontinence which you haven’t mentioned – but people often conceal that symptom in early stages and fail to mention it later out of some sense of delicacy…

  6. Hi Laurie,
    I see he tested negative for lead & mercury. What about arsenic? aluminum? mercury? carbon monoxide? On Wikipedia it says, “Chronic exposure to low levels of carbon monoxide can lead to depression, confusion, and memory loss.”

  7. Hi my name is jen and i have been through this with my father..this is crazy..he is laying in a bed at a skilled nursing facility in a comatose state with normal vital signs..he hallucinates when alert…it all started with an infection on his foot..we had him treated with IV antibiotics for 18 weeks, they didnt work and we had to amputate. The neurologist thinks he has sepsis encephalopathy..which is brain damage from the infection going to his brain and the use of all meds during his hospitalization..I am so sad..he is 70 and just retired and would love some guidance.

Leave a Reply

Your email address will not be published.