Posts Tagged ‘Oliver Sachs’

Six clinical triumphs and a tornado

March 17, 2017

I had me a wonderful day

I was keeping the blindness at bay

And I helped stop the shakes

Oh, the difference it makes!

To start steroids without a delay.

Synopsis: I’m a Family Practitioner from Sioux City, Iowa. In 2010 I danced back from the brink of burnout, and honoring a 1 year non-compete clause, traveled and worked in out-of-the-way places in Alaska, Nebraska, Iowa, and New Zealand. After three years working with a Community Health Center, I went back to adventures in temporary positions until they have an Electronic Medical Record (EMR) system I can get along with. Assignments in Nome, Alaska, rural Iowa, and suburban Pennsylvania stretched into fall 2015. Since last winter I’ve worked in Alaska and western Nebraska, and taken time to deal with my wife’s (benign) brain tumor. After a moose hunt in Canada, and short jobs in western Iowa and Alaska, I am working in Clarinda, Iowa. Any identifiable patient information has been included with permission.

I can’t write about particular patients but I can write about clinical trends.

Sometimes I suspect Parkinson’s in the first 60 seconds after I meet a patient. I note a shuffling gait with arms that don’t swing, a face with limited movement, and a quiet voice lacking in music.  After the patient finishes their concerns,  I ask about shrinking handwriting size and loss of sense of smell.  Then, if the patient lacks the characteristic resting tremor, I’ll hold the patient’s hand.  If I feel the muscles between the thumb and forefinger quivering, I strongly suspect the disease.

No single sign or symptom serves as a gold standard. Without a lab test for Parkinson’s, the diagnosis frequently relies on response to the medication Sinemet, (levodopa and carbidopa).

Parkinson’s always progresses, but the rate varies. No matter what stage I find the patient in, I tell them that we have no medication that will slow disease progression, but we have a whole sequence of drugs for the symptoms.  Most elect to try the meds, a few don’t.

I love seeing a person in the hour or two after their first Sinemet dose, especially if I find the family close at hand. It really deserves the term, awakening, the title of the Oliver Sachs book and Robin Williams movie about the development of the drug.

I have picked up more than one case per week here.

I’m also finding an inordinate number of people with polymyalgia rheumatica (PMR), also called giant cell arteritis or temporal arteritis. For unknown reasons, worse with advancing age, occasionally a person’s body will attack the arterial lining with very large immune cells.  As a result, people feel terrible, lose strength in their shoulders and hips, and get severe morning stiffness lasting more than an hour.  The symptoms can sneak up over the course of months, or ambush over the course of days.  I ask if their jaw gets tired while chewing.  I feel the arteries over the temples; once in a while I feel hot, ropey spots on the scalp, where the blood vessel pulses .  We use two non-specific blood tests, the C-reactive protein (CRP) and the erythrocyte sedimentation rate (sed rate or ESR), which help make the diagnosis.  The conclusive test is the temporal artery biopsy, but only if it’s positive, which it never is.

Blindness ranks as the most feared complication of the untreated PMR; the artery that supplies the retina can clot off. Thus if I have strong suspicion I start steroid therapy promptly.  A quick response helps make the diagnosis.

Last Tuesday I had the wonderful experience of seeing more than one Parkinson’s patient and more than one PMR patient in their initial positive response to therapy.   Bethany picked me up at the hospital, and in the time it took for me to walk out the front door and get into the car the sky darkened so fast that the street lights came on.  As we drove to the gym I started to recount my day full of successes, but halfway there our phones interrupted with the announcement of a tornado warning.  We continued on, but the noise of the hail drowned out my words.  Three blocks later we arrived at the gym just as the tornado sirens started.

We sat on a bench inside and watched the vacant pool while I finished recounting my 6 clinical triumphs in one day.

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Double vision, remembering Wernicke’s without Korsakoff.

December 15, 2015

So double, the patient did see
That he came to the clinic to me
I think, I’m not sure
I can come up with a cure,
Just a shot of Vitamin B

Synopsis: I’m a Family Practitioner from Sioux City, Iowa. In 2010 I danced back from the brink of burnout, and honoring a 1 year non-compete clause, travelled and worked in out-of-the-way places in Alaska, Nebraska, Iowa, and New Zealand. After three years working with a Community Health Center, I am back having adventures in temporary positions until they have an Electronic Medical Record (EMR) system I can get along with. I spent the winter in Nome, Alaska, followed by assignments in rural Iowa. The summer and fall included a funeral, a bicycle tour in Michigan, cherry picking in Iowa, a medical conference in Denver, working Urgent Care in suburban Pennsylvania, and Thanksgiving in Virginia. Right now I’m in western Nebraska. Any patient information has been included with permission.

I attended a patient here with double vision, and I had good cause to think back to another patient I’d cared for out in the Alaska bush. On physical exam, his inability to keep his eyes looking in the same direction struck me as nothing short of dramatic. In the next week, the mystery unfolded. With neurologic consultation and MRI, he turned out to have Wernicke’s encephalopathy, a thiamine (one of the B vitamins) deficiency from excessive alcohol consumption. A vitamin shot or two cured him, and the experience frightened him into sobriety.

I recounted my experience to the patient here. Before firmly establishing a diagnosis, I started thiamine therapy, first by injection and then by mouth. As we have no MRI yet, and the neurologist comes only once a month, I set up both. I arranged those things with less desperation because we have wonderful weather, with services accessible by highway. And then I got on the Internet to research Wernicke’s encephalopathy.

They taught us in med school to look for confusion, double vision, and ataxia (loss of position sense), but in fact later research showed that only 10% of cases include all three parts of the triad. So often does Wernicke’s go with Korsakoff’s psychosis or amnesia that doctors tend to speak of Wernicke-Korsakoff as one word. The diagnosis comes up much more often in autopsy findings than in clinic, indicating we miss all but the worst cases.

Without saying where or when, I told the patient here about my experience with a case of Korsakoff’s, where the patient got stuck in one day, and, despite 3 months therapy for another illness, never got past it.

The patient agreed with me that Korsakoff’s would be the worst complication of alcoholism, more frightening than death from cirrhosis of the liver.

(Oliver Sachs, the brilliant neurologist-author, wrote a detailed account of a patient with Korsakoff’s in his book, The Man Who Mistook His Wife for a Hat.)