Every disease needs a patient advocate, and NMDA receptor encephalitis has found its spokesperson in Susannah Cahalan. For several years prior to and during the onset of her illness, Cahalan worked as a journalist at the New York Post, and she tackles the book with the intensity, readability, and bluntness expected of a Post journalist. Here, she finds the balance between being biographical and educational without becoming laden with facts or burdened by irrelevant detail — it is an engaging read.
In Brain on Fire, Cahalan describes her battle with NMDA receptor encephalitis, a condition first well described by neurologist Josep Dalmau, MD, PhD, and colleagues in 2007. The condition, which is is still not widely and commonly recognized, even among physicians, is an acute autoimmune encephalitis caused by immune-mediated attack against NR1- and NR2-subunits of the glutamate NMDA receptor. NMDA encephalitis is often associated with ovarian teratoma when diagnosed in young women, though it can present unaccompanied by such a teratoma — as in Cahalan's case.
Cahalan describes her entire experience with the illness, from the earliest subtle manifestations of personality change, through the challenges of diagnosis and the acute and devastating phase of the illness. She became amnestic (as most patients do), but only for one month when she was hospitalized; she recreates this most difficult one-month period — the height of her illness — based on interviews and medical records, and goes on to recall in vivid detail the many stages of her recovery.
Essential to any description of NMDA receptor encephalitis are its psychiatric manifestations. Cahalan describes forthrightly the profound effects she experienced across a wide scope of her personality and functioning. Her descriptions of her multiple layers of recovery are especially insightful, from the obvious physical changes to the more subtle and prolonged healing of her psyche. The full spectrum of the psychiatric phenotype in NMDA receptor encephalitis has not yet been fully elucidated, making her descriptions all the more intriguing to readers who are physicians and scientists.
The reader can easily relate to her frustration as she seeks a diagnosis, seeing multiple physicians in multiple specialties, all without a clear answer, while the disorder progresses.
Likewise, physicians will appreciate the difficulties inherent in diagnosing NMDA receptor encephalitis — its symptoms can span multiple specialties, and its presenting symptoms often fall between the cracks of traditional medical diagnostic paradigms. Cahalan describes her misdiagnoses with great care. While the source of the physicians' bias could be debated — her age or gender? — the message is clear: in the absence of a clearly definable diagnosis, at least one of her physicians was quick to assume that she must be hiding a substance abuse problem. The risk for inherent bias is always present in each patient encounter, and conditions such as NMDA receptor encephalitis perhaps amplify such risk owing to its odd presentation; certainly the book reinforces skillfully the importance of acknowledging and removing individual bias in patient-provider interactions.
Perhaps most poignant for physician readers is the glimpse that Brain on Fire provides into the mind of our psychotic or delirious patients — those for whom we have strong memories, whether as trainees or hospitalists; who we have managed acutely, symptomatically in the height of their illness; but with whom we so rarely have the opportunity for long-term follow-up.
Many seasoned physicians, after encountering their first case of NMDA receptor encephalitis, quickly become haunted by memories of patients in years past, prior to the recognition of this disorder — or the other similar autoimmune encephalitidies that have been identified in recent years. Such is the fate of all physicians in the practice of medicine as new discoveries are made, of course, but it seems that the sudden, dramatic, and awful decline that NMDA receptor encephalitis inflicts on its victims makes an especially indelible impression. Equally dramatic, albeit often slow, is the recovery from the condition, as Ms. Cahalan describes so intricately, and with the honest insight that could only come from a patient who suffered from the disease.
While certainly a must-read for all health care providers, the book may well be most valuable as a resource for the families and friends of newly diagnosed patients. By describing both the subtle and dramatic changes from the eyes of the patient, from the earliest symptoms through late recovery, the book accomplishes what cannot be done at the bedside in five or even thirty minutes: it personalizes the disease; it brings the diagnosis from abstraction to reality, accurately portraying the desperation of the acute phase of the illness. Perhaps most important, it gives hope for recovery, but not unqualified by the long duration of the recovery period and the effort required to maximize recovery. As Cahalan observes, the disease exerts a tremendous toll on the patient caregivers, and her book provides a lens through which caregivers can view the behavior of an acutely ill patient.
Dr. Clardy is an autoimmune neurology fellow at the Mayo Clinic in Rochester, MN.