Cui bono

Maria Popova’s Brain Pickings is a very interesting blog with articles and links relevant to what might be called popular neuroscience. As I’ve mentioned several times on this blog, however, one of my pet peeves with respect to some popular (and even academic) neuroscience is what Peter Hacker calls the mereological fallacy–the ascription to people’s brains properties that really apply to people. For example, brains don’t think; people think. Brains don’t see; people see.

As described by Hacker, the mereological fallacy applies to psychological predicates–feeling, thinking, knowing, perceiving, etc. A related practice, and a very common one (or at least, I notice it all the time), is the tendency to assert that something is good for our brains when it would be more appropriate to state that it is good for us, and above is a example of what I mean. It’s a 4 minute TED talk on the benefits of playing a musical instrument, and you can see in the title that the benefits supposedly accrue to the brain. The talk goes through some fMRI and PET evidence that many brain regions are involved in playing music, that this large-scale activity exceeds what is seen in other activities such as drawing, that it is associated with changes in brain structure, etc. Those are interesting findings vis a vis the neural correlates of musicianship, but are they really benefits? As a patient / lay person / citizen / non-neuroscientist, who cares that playing music is associated with certain patterns of fMRI activity? Or with enlargement of the corpus callosum? The more relevant benefits cited in this talk principally involve improvements in subjects’ memory and executive functions. Now those are potentially important findings, which suggest real benefits to us as people (beyond the obvious ones of giving pleasure and relaxation to ourselves and those listening).

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Involve the Palliative Care Team Early

burst-suppression

Here’s a sad story about a family’s struggle with their young son’s fatal ordeal with New Onset Refractory Status Epilepticus (NORSE). NORSE is basically super-refractory status absent an obvious precipitating injury such as encephalitis. The main theme in this account is that the family felt under-informed along the way and then blindsided when it came time to discuss a transition to palliative care.

That frequent and clear communication is both imperative and fraught with challenges will be well-known to most everyone reading this. What I wanted to highlight from this article is actually the follow-on commentary by two neurocritical care physicians, who emphasize that palliative care shouldn’t be reserved for the situation where the patient is on the verge of death. Too often, our palliative care colleagues are consulted when there appears to be no viable means of curing the patient, death is imminent, and assistance is needed with terminal extubation and/or withdrawal of other life-sustaining interventions. However, and this needs to be done with sensitivity so as not to take away the family’s hope, earlier involvement of the palliative care team can be quite beneficial. The palliative care team can recommend measures to ensure the patient’s comfort even while full-on efforts to reverse the condition are underway. They can help clarify the goals of care, ensuring that the patient’s care plan comports with his values. And, if a transition to purely palliative care is eventually desired, it’s helpful for the relationships to have been established earlier in the hospital course and not at the last minute.

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Brain Death Resources

In light of the documented variability in the conduct of brain death evaluations around the country, and the ultra-high stakes, it’s extremely important for residents to know how to do this correctly. I recently became aware of a brain death toolkit published by the neurocritical care society; I added a link to it in Clinical Neurology Resources–>Neurocritical Care in the menu above. The toolkit has lots of helpful information, including links to the relevant guidelines, webinars, simulated video demonstrations, and an online training course developed by the Cleveland Clinic. This course requires a free registration and it’s very good; I highly recommend it. At the least, take the pre-test; if you struggle at all with those questions, they  you should definitely proceed with the course . . .

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Serotonin Syndrome and Ocular Flutter

Serotonin Syndrome is an important entity for residents to be aware of. It’s a toxicity resulting from the use of serotonin-selective reuptake inhibitors and similar drugs, and interactions of those drugs with others such as tramadol and possibly linezolid. The recreational drug MDMA (ecstasy) can also cause it. Manifestations include delirium, rigidity, myoclonus, fever, and life-threatening autonomic instability. Treatment includes discontinuation of the causative agents, benzodiazepines to reduce agitation and help normalize the vital signs and, if necessary, cyproheptadine.

This week’s New England Journal of Medicine has a video of such a patient manifesting ocular flutter in the context of serotonin syndrome. (The link takes you to an image and description of the disease–you have to click the little thumbnail image at the bottom right in order to launch the video). The eye movements are in all directions, so I think it might just as well be called opsoclonus. More examples of these and many other neuro-ophthalmological findings are available at the Neuro-Ophthalmology Virtual Education Library.

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Damn It, Basal Ganglia by Radiolab

Challenge case! Listen to this 12 minute Radiolab podcast and see whether and how fast you can make the diagnosis. The picture below provides a clue. The next paragraph has the answer, so don’t read it until you’re ready.

DeGaper.png

Continue reading

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Robin Williams’s Lewy Body Disease

Lewy Bodies

Robin Williams, who committed suicide in 2014, turned out to have Lewy Body Disease. His widow has written a poignant and informative essay on their struggles with the disease in the current Green Journal.

I think these kinds of accounts can be quite valuable. It’s one thing to study symptoms, signs, pathophysiology, treatment, and prognosis in a reference book or article. But to really refine the diagnostic art, one must become acquainted with the kinds of stories that patients and family members tell. After all, patient don’t usually recite a bulleted list of classic medical symptoms: Asosmia, resting tremor, accelerated forgetting, etc. They tell a story in their own words and it’s our job to parse the story and try to fit it into a medical frame.

We all have have heard a number of stories that is limited by our experience–a relatively small (but rapidly growing) number for residents and a relatively large number for senior clinicians. These kinds of essays can, in just a few minutes, provide memorable information that adds to our differential diagnostic abilities.

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Articles of Neurological Interest

I have a long and growing list of “things to maybe blog about”, and definitely too little time to cover it all. Following are some interesting articles that I’ve read recently (i.e. in the last year) that may be of interest to neurologists. Enjoy!

‘You Want a Description of Hell?’ OxyContin’s 12-Hour Problem.  An informative L.A. Times investigation of the regulatory, marketing, and clinical history of OxyContin in the context of the growing public health problem of narcotic and heroin abuse.

Lost for Words. I really like reading popular descriptions of neurological diseases and the patient experience. They provide a richness that is usually absent from scientific writings and is helpful for recognizing less common diseases. This Atlantic piece describes primary progressive aphasia.

The Neuroscientist who Lost Her Mind. This is a briefer New York Times piece by the director of the Human Brain Collection Core at the National Institute of Mental Health. She describes her experience of having brain metastases.

Why I Hope to Die at 75. This Atlantic article is by Ezekiel Emanuel, a Penn oncologist and bioethicist. The title is a little misleading in that he doesn’t necessarily want to drop dead precisely on his 75th birthday but rather wants to transition at that time to a comfort-only approach to his medical care, even for easily treatable conditions. It’s a very interesting and provocative piece.

Are You Ready for a Glorious Sunset? If the above article wasn’t provocative enough for you, try this Freakonomics podcast: What if, when faced with a serious illness toward the end of life, your insurance company offered you a cash reward to forgo (expensive) life-prolonging care in favor of palliative care?

A Racial Gap in Attitudes Toward Hospice Care. A short, but informative, New York Times piece on the differing ethnic perspectives about hospice and end of life care.

Balancing Faith and Science in the I.C.U. A loosely-related theme to that above.

Hipaa’s Use as Code of Silence Often Misinterprets the Law. This New York Times piece examines several misunderstandings about the Health Insurance Portability and Accountability Act, or HIPAA.

Wastebasket patient. This essay is from the humanities section of the green journal. It provides a highly valuable lesson that one can sometimes make a difference even in the seemingly least promising cases. It’s a particularly important point for residents, bombarded as they are with consults of varying “appropriateness”.

The digital doctor: hope, hype, and harm at the dawn of medicine’s computer age. This one’s a book–one I enjoyed a great deal and highly recommend. It’s by Dr. Robert Wachter, a hospitalist at UCSF and one of the founders of that specialty. He writes about many facets of medicine that have been affected by computerization, especially electronic medical records. Many are quite obvious, such as the frustration of trying to input orders, clicking boxes, etc. Some are non-intuitive. One example: It used to be that medicine teams made a daily pilgrimage to the radiology reading room in order to review all of their patients’ chest x-rays, etc. Now that the images are available on PACS, this happens much less often. More generally, there is less interaction among the professionals caring for patients as each works in his/her computer-based silo–no more bumping into each other while looking for the same paper chart on the unit. This is just one of many interesting and important discussions of how technology is changing our profession.

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Educational Resources

Preparing for the maintenance of certification exam, I came across some very good educational programs on the AAN’s website. I think these would be good for resident education generally, and particularly for RITE preparation. They are free for AAN members, and I’ll add links to them in a new “Board Review” menu above.

The first program is called NeuroLearn. These are slide presentations on various topics, with audio narration and some interactive features such as built-in multiple-choice questions. They are like those really good grand rounds talks that provide a good overview of a topic without getting to much into the weeds of a particular line of research. I particularly recommend the following:

  • The Brachial Plexus. I found this to be one of the best presentations I’ve seen of this somewhat confusing material.
  • Interpretation of the Normal Adult EEG: Normal Patterns and Common Artifacts. A great primer for the neurology resident.
  • Cervical Spondylotic Myelopathy. J.D. Bartleson of the Mayo Clinic narrates this one, and he’s very engaging. He includes some videos of patient examinations and exam techniques, which are helpful.
  • Interpretation of Autoimmune Neurological Antibody Profiles. Not quite as engaging as the ones above, but still pretty good.
  • Paraproteinemia and Neuropathy. This is an excellent one–makes a sometimes confusing topic very clear.

The other program is NeuroSAE (self-assessment exam). These are question banks, mostly covering general neurology topics. There are topic-specific ones as well, covering epilepsy, neuromuscular, and stroke. The best way to improve test scores (as opposed to the best way to become a better neurologist) is to take tests. These questions are very similar to the questions in the back of Continuum (itself another great resource). I set it up to give feedback (i.e., explanations) after each question. You can also have it defer all of the feedback to the end and even have it time the test for you. Another cool feature is that works just as well on a smartphone or tablet as on a PC. I would sometimes start a test at the office and then work on it a little more at home and it did a great job of picking up right where I left off.

I found that it takes a while to answer each question and carefully read the explanation and maybe look up something to dive deeper into the topic. When I actually sat down to take the re-certification exam, I breezed through the 200 questions because in the real test, you don’t get a lengthy response that you have to read through after each answer!

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Nevada, Brain Death, and the AAN Guidelines–part 2

The last post discussed the first concern that the Nevada supreme court had regarding the American Academy of Neurology’s (AAN) guidelines on brain death–that they may not qualify as an “accepted medical standard” as required by the Uniform Determination of Death Act (UDDA). Now we turn to their second concern (starting on p. 17):

[W]hatever their acceptance generally, the briefing and testimony do not establish whether the AAN guidelines adequately measure the extraordinarily broad standard laid out by [Nevada’s brain death statute], which requires, before brain death can be declared under the UDDA, an “irreversible cessation” of “[a]ll functions of the person’s entire brain, including his or her brain stem . . .  A cursory review of medical research raises concerns about brain death testing comporting with [Nevada law]. (Emphasis added)

The medical research they refer to invokes some of the arguments discussed here previously, in the context of Jahi McMath’s case. For example, the maintenance of normothermia (actually a prerequisite for declaring a person dead using neurological criteria) and euvolemia (i.e., the absence of diabetes insipidus) implies intact hypothalamic-neurohypophyseal function. Interpreting the UDDA literally, a patient with any such remaining brain function could not be determined to have died. Needless to say, if this strict interpretation were widely adopted, it would be highly unsettling to now long-established practices regarding individuals with total brain failure (itself a misnomer) and heart-beating organ transplantation. Neither the AAN guidelines, nor the Harvard criteria, nor any other protocol that I’m aware of would satisfy the requirements of the UDDA.

So how did we get here? In the abstract, I think that the UDDA was based on an older conceptual understanding of brain death. Subsequent philosophical scrutiny and empirical data discredited that understanding in favor of different conceptual underpinnings, but the law hasn’t changed to reflect that. And now the Nevada supreme court has just highlighted the discrepancy.

Regarding the older conceptual understanding, consider the 1968 Harvard report: “Irreversible coma has many causes, but we are concerned here only with those comatose individuals who have no discernible central nervous system activity.” (boldface added). Then, in their landmark 1981 publication titled Defining Death, the President’s Commission further elaborated (starting on p. 32) what is known as the “whole brain” concept of life and death. This is the proposition that the brain functions as an integrator for all bodily functions and that when the whole brain is destroyed, somatic integration is lost and the person is dead. This is true even if modern technologies (i.e. ventilators) mask the evidence of death by allowing (for what was then thought to be no more than a few days) continued heart beating and other physiological activities.

Interestingly, the President’s Commission seemed to be aware even then that their “whole brain” formulation may not be strictly true. In a footnote (#4), they quote Dr. James Bernat, et al.: “. . . When the respirator maintains the organism, it is questionable whether there is complete and irreversible loss of the functioning of the entire brain. But this is a question to be settled by empirical inquiry, not by philosophy.” (Emphasis added).1Even more interesting is that the Commission went on to explicitly reject the need for more philosophical analysis: “While it is valuable to test public policies against basic conceptions of death, philosophical refinement beyond a certain point may not be necessary. The task undertaken in this Report, as stated at the outset, is to provide and defend a statutory standard for determining that a human being has died. In setting forth the standards recommended in this Report, the Commission has used ‘whole brain’ terms to clarify the understanding of death that enjoys near universal acceptance in our society . . . Further effort to search for a conceptual ‘definition’ of death is not required for the purpose of public policy because . . . the ‘whole brain’ formulations provide a theory that is sufficiently precise, concise and widely acceptable.” (Emphasis added). That there have been multiple recent court cases on this issue, including the Nevada decision, would seem to belie these claims. To their credit, the President’s Council on Bioethics, in their 2009 re-analysis of this issue, took the opposite position: “At the outset, it is important to note [that we] reject the idea that death should be treated merely as a legal construct or as a matter of social agreement. Instead, [we] embrace the idea that a standard for determining death must be defensible on biological as well as philosophical grounds.” And indeed, as discussed in a previous post, there came to light over time some good empirical reasons to believe that the entire brain does not cease to function in such cases and that the brain is not, in fact, necessary for integrated somatic functioning.

In their 2009 re-analysis of brain death, the President’s Council on Bioethics concurred with this conclusion and formulated instead what they felt to be a more compelling philosophical case–that an organism dies when it can no longer perform its “fundamental vital work”, which in the case of humans they identified with consciousness and breathing.2In my view, and despite the Council’s protestations to the contrary, this has a religious tinge to it, evoking the soul and the breath of God. One of these days I’ll get around to posting some religious perspectives on brain death. However, this change in the philosophical underpinnings of the brain death concept was not accompanied by a corresponding change in the law, setting the stage for the Nevada supreme court’s recent decision.

It’s important to note that the Nevada supreme court did not rule that the AAN guidelines aren’t a medically accepted standard or that following them does not establish whether a person’s entire brain has lost functioning. Rather, they ruled that the lower court hadn’t established those facts and ordered it to consider those issues further. Meanwhile, Ms. Hailu has died according to cardiopulmonary criteria, which might moot the case. But even if Nevada doesn’t adjudicate this case further, their supreme court ruling could be cited in future cases of contested brain death determination. It seems that what is needed is an update to the UDDA that would incorporate the current philosophical understanding of the concept of brain death and establish a national standard, as suggested by Choi, et al., for determining that a person has died according to neurological criteria. And the specific procedure they recommend? The one described in the AAN guidelines!

Notes / References   [ + ]

1. Even more interesting is that the Commission went on to explicitly reject the need for more philosophical analysis: “While it is valuable to test public policies against basic conceptions of death, philosophical refinement beyond a certain point may not be necessary. The task undertaken in this Report, as stated at the outset, is to provide and defend a statutory standard for determining that a human being has died. In setting forth the standards recommended in this Report, the Commission has used ‘whole brain’ terms to clarify the understanding of death that enjoys near universal acceptance in our society . . . Further effort to search for a conceptual ‘definition’ of death is not required for the purpose of public policy because . . . the ‘whole brain’ formulations provide a theory that is sufficiently precise, concise and widely acceptable.” (Emphasis added). That there have been multiple recent court cases on this issue, including the Nevada decision, would seem to belie these claims. To their credit, the President’s Council on Bioethics, in their 2009 re-analysis of this issue, took the opposite position: “At the outset, it is important to note [that we] reject the idea that death should be treated merely as a legal construct or as a matter of social agreement. Instead, [we] embrace the idea that a standard for determining death must be defensible on biological as well as philosophical grounds.”
2. In my view, and despite the Council’s protestations to the contrary, this has a religious tinge to it, evoking the soul and the breath of God. One of these days I’ll get around to posting some religious perspectives on brain death.
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Nevada Supreme Court Questions AAN Brain Death Criteria

Confronted with just about any clinical question, my general practice is to first1After proceeding through the traditional neurological formulation, of course. consider whether there is high-quality evidence to guide decision-making. The best place to search for such evidence is in clinical practice guidelines that summarize and criticize the relevant literature and offer recommendations for specific clinical scenarios.2Of course, practice guidelines exist for only a fraction of the scenarios that we encounter, but almost any clinical question can be widened out to some general case. The evidence regarding management of that general case can then serve as a starting point for a decision regarding the specific situation at hand. For questions regarding acute stroke treatment or prevention, I think that the American Stroke Association guidelines are are the most authoritative resource. When it comes to the determination of death by neurological criteria, the American Academy of Neurology (AAN) guideline is the definitive document. Or so I thought.

The case, as so many of these are, is tragic: About a year ago, 20 year old Aden Hailu presented to the ED for acute abdominal pain. No cause was apparent, and she underwent an exploratory laparotomy. The procedure was complicated by hemorrhagic shock, which caused severe anoxic brain injury. About two months later, she was determined to have fulfilled neurological criteria for death and hospital staff sought to discontinue physiological support. Her father petitioned the courts to prevent hospital staff from doing so, but the district court ruled in the hospital’s favor because the medical testimony showed that the AAN guidelines regarding the determination of death by neurological criteria had been followed.

The Nevada supreme court reversed this decision and remanded the case back to the district court for further exploration of two crucial questions related to the Uniform Determination of Death Act (UDDA) that they felt were insufficiently addressed at the district level. I’m going to address the first question here and the second in a follow-up post.

Before taking up the court’s findings, a quick word about the UDDA: The purpose of the UDDA is to promote uniformity in the determination of death, especially when determined by neurological criteria, so that a person determined to be dead in one state would not be considered to be alive in another. All 50 U.S. states have either adopted this law or passed their own, essentially identical, one. The UDDA states that “An individual who has sustained either (1) irreversible cessation of circulatory and respiratory functions, or (2) irreversible cessation of all functions of the entire brain, including the brain stem, is dead.” Furthermore, the determination of death “must be made in accordance with accepted medical standards” and applied and construed in a manner “uniform among the states which enact it.”

The Nevada supreme court found that the district court and the hospital failed to demonstrate that the AAN guideline on brain death is an “accepted medical standard”. They cite evidence that practices regarding the determination of death by neurological criteria actually vary widely in the U.S. (here’s an even more recent report on that point) and they found that “. . . extensive case law demonstrates that at the time states began to adopt the UDDA, the uniformly accepted medical standard that existed was the then so-called Harvard criteria.” The Harvard criteria refer to a seminal 1968 report titled, A Definition of Irreversible Coma: Report of the Ad Hoc Committee of the Harvard Medical School to Examine the Definition of Brain Death. It’s a very important source document for anyone interested in brain death; unfortunately, I couldn’t find a free, full-text link. The AAN guideline comports closely with the procedures described in this report, with two exceptions: The Harvard report considered an isoelectric EEG tracing to have “great confirmatory value”, whereas the AAN guideline states that “In adults, ancillary tests are not needed for the clinical diagnosis of brain death and cannot replace a neurologic examination.” Second, the Harvard report states that “All of the above tests shall be repeated at least 24 hours later with no change”, whereas the original 1995 AAN guideline recommended a confirmatory examination at 6 hours and the 2010 update is silent on this point. 

That practices regarding the determination of death by neurological criteria vary widely is quite concerning. It is therefore somewhat ironic that the AAN guidelines, intended in part to bring uniformity to this endeavor, may not withstand scrutiny. Complicating matters is the fact that Ms. Hailu has since satisfied cardiopulmonary criteria for having died, so I don’t know if the district court is going to consider the issue moot or continue to adjudicate it. Regardless, this case should prompt neurologists and hospitals to examine their protocols for making brain death determinations.

Notes / References   [ + ]

1. After proceeding through the traditional neurological formulation, of course.
2. Of course, practice guidelines exist for only a fraction of the scenarios that we encounter, but almost any clinical question can be widened out to some general case. The evidence regarding management of that general case can then serve as a starting point for a decision regarding the specific situation at hand.
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