26. The Prognosis

November 07, 2020


How quick can a disease get to you? How long do you have? 

I had seen almost all of my inpatients that morning, and had left the hardest one for last.

Arnold Sanders, 57 years old, came in with severe abdominal pain, nausea and vomiting. He was admitted in the ER a couple of days before for a likely bowel obstruction, and, as expected, an abdominal plain film showed distended bowel loop with air-fluid levels. Along with that, Arnold had a history of losing a lot of weight for the past 6 months, increasing weakness and fatigue. No surprise the first word that came to mind was Cancer. Probably a colon cancer that was now obstructing his intestinal lumen. 

It turns out, that was not his case. His upper endoscopy and colonoscopy were completely clean. No lesions at all, and nothing that could explain his pseudo-obstruction. After his condition was stabilized and managed with medications, Mr. Sanders was sent to our floor. We couldn’t explain his condition yet and that was what made him the hardest and last one. 

‘Hi, Mr. Sanders! How are you feeling today?’

‘Same thing, Dr. Diaz. My stomach feels a little better, but the pain is still there a little. And I still feel very weak.’

It had been 2 days since I got his case. His abdominal symptoms were fading thankfully. But he complained of feeling weak, something that we all thought it could be due to his vulnerability, weight loss and even age. But that day, I noticed that maybe I wasn’t being thorough enough in his case. It was time to change that.

‘What do you mean when you say you feel weak, Mr. Sanders?’

‘It’s difficult for me to walk, I can’t hold things for too long, not even a fork to eat.’

‘When did this start?’

‘Well, it’s been going on for about a year now. But it’s getting worse.’

Indeed, he had a lot of difficulty sitting in the bed, I never saw him walking in the hospital, and the staff had to help him walk to the bathroom and back.

‘I wasn’t like this, you know. I had a store I worked in every day, moving packages and getting the work done.’

That wasn’t usual aging. I decided to go for a neurological exam. He had decreased strength in all four limbs. Some of his muscles in his thighs seemed atrophied, which made me think of something peripheral as a problem. Maybe neurological, maybe muscular, maybe paraneoplastic. But then his arms were stiff, and spastic, which could suggest a central problem. So I went to test his tendon reflexes. Again: some hyperreflexia, some hyporeflexia. Damn it.

‘Arnold, why do you think you lost so much weight?’ Something I should have asked sooner.

‘Oh, I don’t eat as I used to. I choke with the food while trying to swallow, so I don’t enjoy it so much.’

‘So this is also a recent problem? Difficulty to swallow?’

‘Yes. All in this past year.’ At this point I was pretty positive he was a neurological patient, with something that could probably even explain his intestinal pseudo obstruction.

‘And did anything else change during this time? Anything at all?’

He thought for a moment. ‘Well, my kids say my voice is changing, but I think that’s probably because of my age, right?’

I thought of how to answer. ‘I am not sure, Mr. Sanders. But I’ll tell you when I find out.’

Then I asked to examine his oral cavity and throat. Nothing of note there, except for his tongue. When he opened his mouth I could see fasciculations all over it.

I was eagerly waiting for the rounds that day, excited to share some new insight into Arnold’s case. Until then, he was just waiting for his body to recover and receiving some nutritional support to get discharged. He would be sent home with a medical appointment scheduled to investigate his case in the outpatient clinic. 

When Lucy arrived, Peter presented his cases first. When my turn came, I started with Arnold.

‘So, I took some time to talk more with Mr. Sanders today. His symptoms of weakness are bothering him a lot. They started a year ago and have been getting progressively worse. He can’t walk well alone, and has difficulty carrying and holding objects. He also associates his weight loss with a dysphagia that appeared in the past year with his other symptoms.’ I could see Lucy’s eyebrows raised, intrigued. ‘And at last, his children noticed changes in his voice.’

‘That changes everything, doesn’t it?’ Dr. Collins asked and we nodded. ‘Did you perform a neurological exam?’

‘Yes. He had atrophied muscles throughout his body, but at the same time some of his upper arm muscles seemed spastic, you know? And I am not sure I examined his tendon reflexes right, because some seemed diminished, and others seemed increased.’

‘I don’t think you got the reflexes wrong, Lisa. Go on, did you see anything else?’

‘His tongue. Had these weird fasciculations.’

‘We should call Neuro. But your presentation already leaves me with a very likely diagnosis in mind. Guesses?’

Before Peter and I could make any guess, Lucy interrupted us.

‘Wait! Let’s do it the way my neurology teachers taught me.’ She winked. ‘Syndrome, Topography then Etiology. What neurological syndrome does he have?’

‘A motor one.’ Peter said.

‘And generalized.’ I completed it.

‘That’s right.’ Lucy nodded. ‘But which kind of motor lesion?’

‘It’s hard to say.’ I answered. ‘He has signs that point to lower motor neuron problems, like the atrophy in some muscles, and signs that point to upper motor neurons, like the muscle stiffness and hyperreflexia in some tendons.’

‘What if he has both?’ Peter asked.

Then everything clicked together in my head. ‘That’s it! He has lesions in upper motor neurons, like cortex or corticospinal tracts, and lower motor neurons from anterior horn.’

‘And that’s topography.’ Lucy smiled. ‘Etiology?’

‘ALS.’ We both said together.

‘Precisely.’ Dr. Colling seemed satisfied. ‘Now, Lisa, ask Neuro for a consult so they can order the workup and confirm the diagnosis.’

‘Ok.’ I wrote it down.

‘If it is Amyotrophic Lateral Sclerosis, that is no great news to give. We will start him on Riluzole to slow disease progression, but it is a poor prognosis.’

After we discussed my other patients, I stayed there for a while waiting for the Neurology resident that would stop by to discuss Arnold’s case with me. He told me it really sounded like ALS and that they would request a nerve conduction test and some other exams. 

All the tests he took ruled out other causes, so Arnold began treatment for ALS in Brooklyn’s Hospital. As soon as he was good enough to go home, he was discharged. 

‘The more time we can give to him at his home, the better.’ Lucy had said.

She was right. It didn’t take him long to get admitted again, with another episode of intestinal pseudo-obstruction. He went to another floor but I stopped by his bed to visit him. He had become dysarthric and needed a wheelchair by then. He smiled sadly when he saw me. And I smiled back.

That’s how quick ALS got to Arnold.

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ALS: Amyotrophic Lateral Sclerosis; Neurod: neurodegenerative; 
SS: signs and symptoms; Dx: diagnosis; UMN: upper motor neuron; 
LMN: lower motor neuron; Tt: treatment.

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