Body of evidence

It's a moderately busy night on internal medicine call. After completing two admissions, you check the admit board and it looks pretty clear, so you contemplate a few sweet hours of REM in the call room with about five hours left on shift. The last hour has been quiet. You kick off your shoes and flop supine onto the too-firm mattress and rubbery pillow, nothing between you and the sweet arms of Morpheus.

Nothing but your pager, that is.

A nurse reports that a patient is becoming progressively more agitated. He is no longer redirectable and aggressively grabbed her arm. He wants to know why his mother is in the room, though she has been dead for decades. The nurse asks you to order some intramuscular haloperidol. You are about to verbally OK the medication, but decide maybe you should think about the patient a little bit more, and drag your sleepy carcass to the computer.

The patient is an 82-year-old man admitted with cellulitis and a history of hypertension, type 2 diabetes, possible Parkinson's disease, and dementia. An alarm is ringing in your head. Parkinsonism, hallucinations, dementia. That rings a bell. Could this be Lewy body disease? And for that matter, why does Lewy even have a body? Inquiring minds need to know.

Dementia with Lewy bodies is named for Frederic Lewy, a German-born neurologist. After graduating from medical school in 1910, he began working in the laboratory of the equally eponymic Alois Alzheimer. In 1912, while studying paralysis agitans (later known as Parkinson's disease), he described findings of eosinophilic inclusion bodies in the neurons of the dorsal nucleus of the vagus nerve. He described them as “spherical, strand-like, and serpiginous forms … that stain bright red with Mann's technique.” He published a book in 1923 in which he detailed the various regions of the brain and described a condition associated with degenerative changes in Meynert and vagus nuclei. These findings would later become known as Lewy bodies, and they continue to be the pathognomonic sign of Lewy body disease.

Interestingly, a year after his exhaustive book was published, Dr. Lewy stopped discussing his pathology findings, although he went on to publish many more papers. Perhaps he didn't realize their great importance, but the exact reason for his abandonment remains unclear. That book was his full body of work on the topic.

Dr. Lewy died in 1950. Eleven years later, an account of similar inclusion findings in two autopsied patients with dementia was published. Kenji Kosaka, a Japanese psychiatrist, described in detail a dementia condition associated with these histopathological findings in 1976. Four years thereafter, he proposed the term “Lewy body.”

Climbing from that historic rabbit hole, you go to see the patient. He is agitated and needs medication, but definitely not haloperidol, which is most likely to cause extrapyramidal side effects and will certainly worsen his symptoms. Instead, you sit with him and try to engage him while you order quetiapine. You also order sleep enhancement measures.

You head back to the call room. The patient is cared for and your curiosity is satiated by the body of evidence. You're ready once again to grab some shut-eye. But there goes the pager again, an admission in a 50-year-old with degenerative muscle weakness. For some reason you already suspect it might be inclusion-body myositis.