Dementia with Lewy Bodies and the Neurobehavioral Decline of Mervyn Peake

OBSERVATION Dementia With Lewy Bodies and the Neurobehavioral Decline of Mervyn Peake

Demetrios J. Sahlas, MD, MSc

ervyn Peake (1911-1968) was an accomplished British artist, poet, novelist, and playwright. He was a prolific and talented illustrator and wrote hundreds of poems, 4 novels, and several plays. His exceptional career was prematurely ended by a neurodegenerative illness variously ascribed to Alzheimer disease, Parkinson dis- Mease, or postencephalitic parkinsonism. However, a detailed review of biographical accounts pro- duces substantial evidence in support of a probable diagnosis of dementia with Lewy bodies, a clinical entity remaining undiagnosed outside specialty dementia clinics. Peake developed signs of parkinsonism and insidious cognitive decline during his fifth decade. A breakdown in his writing style has frequently been cited as reflecting his encroaching dementia. Visual hallucinations are portrayed in sketches, and together with paranoid delusions are apparent in poetry composed during his illness. His deterioration was progressive and punctuated by well-described episodes of confusion and psychosis. His occasional preservation of insight is poignantly captured in drawings of figures with dunce caps or pointed heads, often with expressions of fear and apprehension etched with an economy of strokes. Peake spent his final years in various psychiatric institutions but continued to exhibit lucid intervals even late into his illness. His tragic deterioration remained undiagnosed at the time, but in retrospect, his progressive dementia with parkinsonism, visual hallucinations, and marked cognitive fluctuations likely represents one of the earliest recognized historical cases of dementia with Lewy bodies. Arch Neurol. 2003;60:889-892

Dementia with Lewy bodies (DLB) is es- MERVYN PEAKE timated to account for 15% to 20% of cases of dementia, based on autopsy data1 and Mervyn Peake was born in Kuling (Lus- epidemiologic studies.2 Wider recogni- han), China, in 1911. His father, a British tion of this disorder has emerged only re- missionary physician, brought his family cently, coinciding with descriptions pub- back to England in 1922. As a young man, lished since the 1980s.3 To date, reports Peake entered art school and first exhib- of prominent historical cases are lacking, ited his work in 1931. He soon estab- although with increasing awareness, such lished a reputation as a talented illustra- cases are more likely to be recognized in tor. He was called up for military service retrospect. A probable diagnosis is appar- in 1940, and while serving in the army, ent in the neurobehavioral deterioration started work on his Gormenghast novels. of accomplished British artist, poet, nov- After the war, he was commissioned to elist, and playwright, Mervyn Peake (1911- document the aftermath of the conflict and 1968), whose celebrated Gormenghast completed a series of heartbreaking sketches novels were recently adapted into a criti- of concentration camp survivors at Belsen. cally acclaimed television miniseries by the He continued to write and illustrate, pro- British Broadcasting Corporation.4 Peake’s ducing many of his finest paintings, draw- persistent attempts at artistic expression ings, and poems during the late 1940s in the face of a relentless, cognitively dev- (Figure 1). He was much admired as an astating illness provide us with a unique art instructor, and during this period also and intensely personal account of a his- scripted several plays.5 torical case of DLB. Peake first showed signs of a myste- rious neurodegenerative illness around From the Sunnybrook and Women’s College Health Sciences Centre, Department of 1956, developing signs of parkinsonism and Medicine, Division of Neurology, University of Toronto, Toronto, Ontario. symptoms of cognitive decline in his fifth

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Figure 1. These examples are taken from some of Mervyn Peake’s earlier work, prior to the onset of cognitive decline. A, Self-portrait. Oil on canvas (National Portrait Gallery, London). B, Illustration for a 1949 edition of Robert Louis Stevenson’s Treasure Island. Published with permission from the Mervyn Peake estate.

gressive, punctuated by well-described episodes of con- Consensus Criteria for a Diagnosis of Dementia 11,12 fusion and psychosis but relieved by intermittent lucid pe- With Lewy Bodies (DLB) riods. He was ultimately diagnosed as having parkinsonism and “premature senility.”8 1. The central feature is progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function. In 1960, neurosurgical approaches for Parkinson dis- Prominent or persistent memory impairment may not necessarily easewerebeingattempted,andPeakeunderwentoneofthese occur in the early stages but is usually evident with progression. procedures in an effort to ameliorate his parkinsonian symp- Deficits on tests of attention and of frontal-subcortical skills and toms. The effects were disappointing. As a last resort, an visuospatial ability may be especially prominent. appointment was arranged with the preeminent British neu- 2. Two of the following core features are essential for a probable, and 1 for a possible diagnosis of DLB: rologist of the day, Sir Russel Brain, but he was likewise un- Fluctuating cognition with pronounced variations in attention and able to assist the ailing artist. Peake spent the next several alertness years of his life in various institutions, treated with neuro- Recurrent visual hallucinations that are typically well formed and leptics and electroconvulsive therapy, until his death in 1968 detailed at the age of 57 years.8 His work has since drawn substan- Spontaneous motor features of parkinsonism tial critical acclaim and is presently the subject of increas- 3. Features supportive of the diagnosis are: Repeated falls ing public interest. His influence on subsequent generations Syncope or transient loss of consciousness of artists and writers has been profound. Neuroleptic sensitivity Systematized delusions DEMENTIA WITH LEWY BODIES Hallucinations in other modalities Depression In 1912, Friederich Lewy described the presence of intra- REM sleep behavior disorder cytoplasmic inclusion bodies in the dopaminergic neu- 4. A diagnosis of DLB is less likely in the presence of: Stroke disease, evident as focal neurologic signs or revealed on rons of the substantia nigra in patients with Parkinson 9 brain imaging disease. Lewy bodies in the midbrain are spherical, eo- Evidence on physical examination and investigation of any physical sinophilic inclusions, differing slightly in appearance from illness or other brain disorder sufficient to account for the those described in the neocortex. Reports of cortical Lewy clinical picture bodies were rare until new staining techniques for ubiquitin were introduced in the 1970s. The occurrence of cor- Abbreviation: REM, rapid eye movement. tical Lewy bodies in cases of dementia was firmly estab- lished by the 1980s.3 Antibodies to ␣-synuclein, which decade.5 Perplexed biographers have alternately specu- immunostain Lewy bodies, have since replaced the use of lated diagnoses of Alzheimer disease,6 Parkinson dis- the ubiquitin stain, and DLB is now classified among the ease,7 or postencephalitic parkinsonism5 after question- spectrum of diseases regarded as synucleinopathies.10 ing a history of viral encephalitis6 or encephalitis lethargica.5 Consensus criteria for the diagnosis of DLB were pub- The disintegration of Peake’s writing style has frequently lished in 1996 and refined in 1999 (Table).11,12 The pro- been regarded as reflective of his encroaching dementia.7 spective sensitivity and specificity of the consensus cri- He also suffered from bouts of depression, which influ- teria for DLB are reported at 0.83 and 0.95, respectively.13 enced his later work. After a stressful period that culmi- Previous retrospective studies yielded comparable speci- nated with a number of disastrous play reviews, his be- ficities (0.87-1.00) but lower sensitivities (0.22-0.89).14 havior started to become erratic, and his wife reported that Hence, although the criteria might not identify every in- he experienced hallucinations.8 His deterioration was pro- dividual harboring the condition, there is high certainty

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Figure 2. Sketches by Mervyn Peake made late in the course of his dementia. A, Figure with dunce cap, a repetitive theme. B, Animal-like floating creatures surround a tormented individual, drawn while Peake was suffering from visual hallucinations. Published with permission from the Mervyn Peake estate.

of a diagnosis of DLB if the criteria are met. Age at onset cial effects of L-dopa in the management of Parkinson dis- generally ranges from 50 to 83 years,15 although autopsy- ease were not discovered until the 1960s, and so treatment verified cases have been documented in patients with ini- in the preceding decade was limited to the use of anticho- tial symptoms at an age as young as 27 and 33 years.16 linergic medications and experimental surgical pallidotomy. The mean survival time is usually comparable with that Later photographs of Peake reveal the stooped posture and of Alzheimer disease.15 inscrutable expression characteristic of parkinsonism. Peake was tormented by recurring visual halluci- HISTORICAL EVIDENCE nations, the early development of which—soon after the onset of his other symptoms—is further suggestive of DLB. Peake’s progressive cognitive decline is apparent in his later Many well-formed and detailed examples are evident in work. His pronounced difficulties with attention and visuo- his sketches (Figure 2B). These hallucinations, together spatial ability are typical of DLB, in contrast with the more with the paranoid delusions he experienced while in the prominent decline in memory function observed in Alzhei- hospital, were incorporated into poetry composed dur- mer disease.11 Commencing with his later illustrations, one ing his institutionalization: biographer observed that, “there is a coarseness of feeling, a lack of sympathy for the persons he portrays.”8 (p219) By Heads float about me; come and go, absorb me; 1958, his drawings were more caricaturelike and later be- Terrify me that they deny the nightmare came “geometric and then almost abstract”8 (p219) in keep- That they should be me, defy me; And all the secrecy; the horror ingwithPeake’sinexorabledeteriorationofvisuospatialfunc- Of truth, of this intrinsic truth tion. In this respect, his case is comparable with those of Drifting, ah God, along the corridors 17 18 Willem de Kooning and William Utermohlen, in whom Of the world; hearing the metal similar changes were noted in artistic ability with advance- Clang; and the rolling wheels. ment of probable Alzheimer disease, likely arising from in- Heads float about me haunted volvement of posterior isocortical association areas, with By solitary sorrows19(p33) resultant deterioration of visuospatial function. Although problems with memory were apparent by Other poems, perhaps indicating auditory as well as vi- 1960, Peake’s “attention span had become so short that sual hallucinations, and letters to his wife reflecting his he could no longer read a story and retain the idea for frightening delusions, are further discussed in a recent an illustration long enough to draw it.”8 (p223) Thus, both biography.5 cognitive domains predominantly affected in DLB were By the time he entered his sixth decade, Peake “was especially involved. Conversely, a tenuous preservation with us only in flashes, and those flashes were often over of insight was captured in drawings of figures in dunce before we had grasped what he had said, or could reply. caps or with pointed heads, often with expressions of fear Most of the time he sat in silence, head bowed.”20 (p37) A and apprehension etched with an economy of strokes remarkable example of the striking fluctuation in his men- (Figure 2A), distinguishing him from artists diag- tal status follows: nosed as having probable Alzheimer disease.18 Peake’s parkinsonian signs and symptoms have been After dinner he was sitting humped on the sofa when suddenly well documented in biographies.8 In his mid 40s, he devel- he made a motion towards Maeve, and as she leaned over him he indicated that he wanted some paper and something to draw with. oped shaking of the hands and was tentatively diagnosed My wife produced a sheaf of quarto typing paper and placed a as having Parkinson disease after evaluation at the National ball-point pen in his swollen hand. We went on talking while he 5 Hospital, Queen’s Square (London, England). The shak- sat with the sheaf of white paper on his knee....Then I noticed ing “spread to his legs as well as his hands” and exhibited that his hand had ceased its normal shaking, and that he was sit- “remorseless progression” over the years.8 (p213) The benefi- ting upright with the paper held firmly, concentrating. For more

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©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 than an hour, he seemed to lose touch with his illness alto- cognitive fluctuations likely represents one of the earliest gether. He covered page after page with wonderful and prepos- recognizable, and possibly best-documented, historical cases terous beasts, leaping, snarling, laughing, cavorting. As soon as of DLB. There is abundant biographical evidence that he one was finished he turned to a fresh page and drew another. We exhibited all of the core features (only 2 of which are re- self-consciously kept up our conversation, although all three of quired for a probable diagnosis of DLB), as well as most of us were watching him in amazement. He appeared unaware of our presence. And then the spell was broken. He dropped the pen, the ancillary features supportive of the diagnosis. and his hand began to shake again.”20 (pp37-38) The burgeoning popularity of Peake’s work serves as a testament to his brilliant expressions of imagination. That This is a compelling description of a dramatic fluc- such a gifted artist succumbed to a dementing illness with tuation in cognition and alertness, which is typical of DLB a predilection for attention and visuospatial function is par- and not other forms of dementia, such as Alzheimer dis- ticularly tragic. His later work stands as a unique and de- ease. There are abundant examples of similar observa- fiant record of his struggle with the disease. Recognition tions in accounts of his illness.5,7,8 of the likely cause of his affliction as his popularity contin- Several features supportive of a diagnosis of DLB are ues to grow may ultimately serve to heighten awareness of also apparent in biographical material of Peake’s neurobe- this distinct and devastating form of dementia. havioral decline. Repeated falls were observed, as “his sense of balance would suddenly desert him, and he would lurch Accepted for publication October 17, 2002. and fall against objects and people.”21 (p204) Moreover, there Dr Sahlas is supported by a joint personnel award from isfrequentreferencetoneurolepticsensitivity,since“although the Canadian Institutes of Health Research, Ottawa, Ontario, the doctors gave him tranquilizers, the tremors in his hands and the Heart and Stroke Foundation of Canada, Ottawa. andlegsincreased.”8 (p218) Thorazineandchlorpromazinewere I would like to thank Sebastian Peake, the artist’s son, and the most common neuroleptics used in the 1950s and 1960s. the Peake family for their interest as well as for their kind per- His wife often ironically observed that the medications doc- mission to reproduce the poetry and artwork contained herein. tors gave him only seemed to make him worse.8 One of his The helpful staff at the Merril Collection, Toronto Public Li- physicians also noted that some of the treatments “aggra- brary, is gratefully acknowledged. Special thanks are extended vated the parkinsonism, rather than improved it.”21 Wors- to Sandra E. Black, MD, for a critical review of the manuscript. ening confusion and exacerbation of parkinsonism are com- Corresponding author and reprints: Demetrios J. Sahlas, mon consequences of neuroleptic use in DLB.12,15 MD,MSc,FRCP(C),DepartmentofMedicine,DivisionofNeu- Peake’s symptoms developed at a younger age than rology, University of Toronto, Sunnybrook and Women’s Col- most patients with DLB, although the duration of his ill- legeHealthSciencesCentre,2075BayviewAve,A421,Toronto, ness was more in keeping with the diagnosis. Through- Ontario, M4N 3M5, Canada (e-mail: [email protected]). out his institutionalization, he was victim to systematized delusions and hallucinations. His occasional bouts REFERENCES of depression intensified over time. In addition, rapid eye movement sleep behavior disorder may have been pres- 1. 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