Brain on Fire
Author: Susannah Cahalan

Background

This illness narrative is the classic account of the horror that is autoimmune encephalitis. Reconstructing the story from the diary entries she made during what she called ‘my lost month of madness‘, the author describes how she lost her ‘rational consciousness‘ as a mysterious disease wreaked havoc with her mental state and challenged the wits of her doctors. But the book is more than just a chronological account of a relatively new and little understood neurological disorder. On one hand, it is a critical commentary on the shortcomings of the patient-doctor relationship, and how this impacts outcomes in healthcare. On the other, it is a delightful exploration of the extreme vulnerability of the human brain, a structure it portrayed as ‘a symphony of 100 billion neurons’ whose cohesion can be marred by ‘only one dissonant instrument’, and as ‘a circuit of Christmas tree lights’ which ‘twinkle brilliantly’ until ‘one blown bulb’ makes ‘the whole strand go dark‘ (pages 41, 60, 72 and 83).

Synopsis

The author provided a very detailed account of her illness which began innocuously enough with minor cognitive symptoms, but rapidly spiralled into a medley of mental symptoms. For example, she describes how her thoughts became ‘tangled‘ in her mind ‘like necklaces knotted together in a jewellery box‘, and ‘just when I thought I had untwisted one, I would realize it was connected to a rat’s nest of others’. She also described a disconcerting psychosis manifesting with such bizarre symptoms as ‘irrational jealousy‘ and hysterical crying spells, and a change in her personality from one characterised by patience, kindness, and courtesy, to one dominated by erratic and ‘barbarous‘ behaviour, such as nudging elderly people out of her way. Some of the most dramatic episodes of her illness occurred whilst she was under the influence of a paranoid hallucination, such as when she attempted to jump out of a moving car ‘headfirst‘. It was however pertinent that even at the worst stages of her illness, ‘when I could hardly recognize myself’, she said that there were still ‘shadows of the real Susannah, a person who cares what her family and friends think, who doesn’t want to cause them pain‘ (pages 39-43, 55-56 and 61-62).

A central theme of the book is the author’s account of her arduous neurological journey which was characterised by the erroneous assumptions and mistaken judgements made by her doctors. For example, the author explored some of the wrong diagnostic labels she was given, from viral encephalitis and bipolar disorder, to post ictal psychosis and schizoaffective disorder, and she was understanding of how these errors were made given her presentation. She however found her other misdiagnoses to be less explicable, and she illustrated this with the example of a doctor who concluded, without evidence, that she was ‘exhibiting the classic symptoms to alcohol withdrawal, and of the neurologist who thought she was just ‘partying too hard, not sleeping enough, and working too hard’. Whilst the author attributed some of these misdiagnoses to hasty deductions, she also blamed the healthcare system for why ‘it took so long for one of the best hospitals in the world’ to make her diagnosis. She was particularly critical of how the system allocates ‘very little time’ for neurologists to assess their patients, a situation which she said is ‘designed to miss cases just like my own – cases that require time and patience and individualized attention‘. She was therefore justifiably concerned that many people with her condition are ‘going untreated, diagnosed with a mental illness or condemned to a life in a nursing home or a psychiatric ward’ (pages 50, 70, 81, 86, 226-227 and 151).

A most enlightening theme of the book is the author’s lucid documentation of the neurological assessments she underwent. In what may pass as a masterclass in the neurological examination for example, she outlined the meticulous cognitive tests administered to her which localised her brain disorder to the right parietal lobe. In detailing this, she recalled that her clock-face drawing test, which assesses procedural memory, was marred by perseverative dysgraphia such that she ‘squished‘ all the numbers on one half of the clock face. She also provided a detailed commentary on the NMDA receptor antibody test, devised by neurologist Josep Dalmau, which clinched her diagnosis; in the process, she explained that the receptor plays a major role in hippocampal learning, memory, and behaviour. Using a similar approach, the author also reviewed her MRI head scan, her video EEG, and her lumbar puncture or spinal tap test which showed progressive elevation of the white blood cells over time. And in documenting her treatment, she outlined the ‘three-pronged’ aggressive immune treatment she received which comprised IVIG, steroids, and plasmapheresis. The narrative also followed her slow and bumpy road to rehabilitation, noting that ‘the stages of recovery often occur in reverse order‘ (pages 16-17, 75, 107, 115, 123, 129-150, 161-162, 165, 176-179, 189-193 and 240).

The book’s exploration of the neurological concepts associated with her illness was also very educational. For example, in explaining the reason she developed profound short-term memory difficulties, she described the hippocampus as ‘a way station’ which passes along ‘patterns of neurons that make up memory‘ to different parts of the cortex, adding that the cortex is ‘responsible for preserving them long-term‘. She also explained that many of her puzzling symptoms were actually manifestations of seizures, and these included lip smacking – ‘as if I was sucking on a candy’; jamais vu – ‘when everything seems unfamiliar‘; and Alice in Wonderland syndrome – ‘viewing the world as if it is bizarrely out of proportion‘. Other strange symptoms she also attributed to seizures were feelings of alienation, euphoria, sexual arousal, religious experience and out-of-body sensations. She similarly explored the pathology behind her psychiatric symptoms, for example when she ascribed her belief that her father was an impostor to Capgras syndrome – a disorder that she said develops when the rational and emotional parts of the brain become incongruent. The book also explained many neurological symptoms such as aphasia, dysarthria, and  phonemic paraphasia, and concepts such as neuroplasticity (pages 42, 58, 77-78, 97, 101, 152-153, 156-158 and 169-170).

The author reflected movingly on the human aspects of her illness journey, noting the wide divergence in the empathy she perceived from her healthcare providers. For example, she criticised the discourtesy of a group of doctors who ‘stared at me as if I were a caged animal in a zoo‘, particularly condemning their leader who ‘introduced my case as if I weren’t in the room‘. She contrasted this with the ‘very personal, heartfelt bedside manner‘ of her neurologist Souhel Najjar who ‘spoke directly to me, as if I was his friend instead of his patient’. The author also highlighted the importance of having caring relationships when facing such devastating diseases, and she illustrated this with how her friends, partner, brother, and parents supported her throughout, without which she believed that the outcome of her illness would have been tragic. Her brother and mother, for example, were instrumental in getting her hospitalised in defiance of her doctor’s wrong diagnoses that she was suffering from alcoholism and depression. The author was therefore full of commendations to her family for their tolerance of her outlandish paranoid accusations and frankly rude behaviours, and she also took comfort that even in her darkest days, she would ‘visibly relax‘ when her partner entered the room (pages 53, 71, 93, 159 and 128).

Opinion

With very short and easy to read chapters, this account of a relatively new, and difficult to diagnose, smouldering brain disease is clear as it charts the author’s progressive decline into irrationality and bizarre manifestations. The author, a journalist, describes the constellation of perplexing symptoms that typify this condition, all the while providing the neurological contexts that explain them. The book is also well-researched as it comprehensively explains how a physical brain disorder may present with diverse psychiatric features. Whilst the chronological description of the buildup of her illness was rather drawn out, it did serve to illustrate the slow and fluctuating evolution of the disease in real time.

Overall assessment

In a great public health service, this book brings to general awareness the existence of a group of disorders which are difficult to diagnose but eminently treatable. The book also discusses the healthcare attitudes that could impair diagnosis and treatment – from cursory attribution of symptoms to system-determined time limitations. The book’s focus on a serious brain disease and its mimics does an invaluable service to healthcare and I recommend it to all doctors.

Book details

Publisher, Place, Year:  Penguin Books, London, 2012
Number of chapters: 53
Number of pages: 259
ISBN: 978-0-141-97534-4
Star rating: 5
Price: £7.99