Music and Mental Illness in Search of Lost Time From "Demonic Possession" To Anti-N-Methyl - Aspartate Receptor Encephalitis

LETTERS/REPLIES
Music and Mental Illness In Search of Lost Time

Hamilton Moses III, MD From “Demonic Possession” to Anti–N-Methyl-D-
Aspartate Receptor Encephalitis
Your editorial in July1 just scratched the surface. Guillaume Sébire, MD, PhD
Neanderthals aside, more recent folklore points to a link
The fascinating discovery of anti–N-methyl-D-aspartate receptor
between specific personality types or temperaments and choice
(NMDAR) pediatric encephalitis creates new connections be-
of instrument among performing classical musicians. These in- tween past and present, but likely identical, syndromes.1,2 There
clude obsessionality (double-reed and organ), promiscuity was first the strange story of a 14-year-old boy admitted in 1949
(brass), hysterical traits and hypochondriasis (conductors and at the Alexian Brothers Hospital (Saint Louis, MO) for frighten-
singers), superstition (strings), and others. Unipolar depression ing behavioral disturbances (original description attributed to Rev.
and bipolar illness are said to be especially common among William S. Bowdern, SJ3). Clinical manifestations included explo-
composers, which may make them similar to writers, as Kay sive episodes of rage, extreme auto- and heteroaggressivity, speech
Redfield Jamison suggests. deterioration, sudden “shouts in unknown tongue,” and bouts of
MacDonald Crtichley and D. A. Henson, in their book contorted postures into seemingly impossible shapes,” propagating
Music and The Brain, mention some of these stereotypes. In vibrations, and even “movements to surrounding pieces of furni-
the early 1970s, Critchley lectured on the accentuation of such ture.”3 The patient recovered soon after an exorcism procedure.
traits in the early stages of dementia and in recovery from This case report, popularized by the famous novel and movie The
aphasia and closed head injury. Those who were at Queen Exorcist, presents striking clinical similarities with most manifesta-
Square at the time will remember several performers with mi- tions of anti-NMDAR encephalitis. Forty years later, a series of 6
boys was reported with a so-called “recognizable syndrome of
nor head trauma from a local orchestra who were disabled by
coma associated with intense bursts of abnormal movements and
behavioral disturbances following a bus accident. Their musi-
long lasting cognitive and behavioral disturbances.”2 Again, iden-
cianship was relatively unimpaired, but affective or behavioral
tically to anti-NMDAR encephalitis, the picture was dominated
abnormalities were severely limiting. Critchley believed they
by loss of language, dementia regression including autistic traits,
were far more affected than patients from other walks of life episodes of extreme agitation, aggressive behavior, and abnormal
having similar injuries. movements such as dystonia and orofacial dyskinesia subject to
It is remarkable how common and true-to-form such as- spectacular paroxysms. Most of these patients also had conscious-
sociations seem to be. Similar folklore is often heard around the ness impairment, autonomic dysfunctions, and occasional seizures.
United States and Europe. In fact, 1 conservatory dean used Paraclinical investigations were unspecific. The recovery phase was
them as a rule of thumb in selecting students. The audition was protracted over months.2 Under antiepileptic treatment, some-
important, but he looked for performers who did not fit the times neuroleptic drugs, and supportive care, but without any ex-
stereotypes for each instrument, as he believed they were more orcism, most patients eventually had full recovery. At this time, an
likely to succeed. encouraging comment in regard to the potential future of this
Creativity is another matter altogether. True creativity in presumed new entity came from an anonymous reviewer who
any domain is reflected by an ability to recognize or produce stated, “I would hazard a guess that most experienced pediatric
something novel. The ability may be fully conscious (and there- neurologists have seen a few cases similar to those described by the
fore a cognitive function). It may also be largely unconscious authors.” Since 1992, this guess has been substantiated by child
neurologists from many countries who kindly reported to me, at
(and therefore an intuitive function). It is usually both cognitive
meetings of the Child Neurology Society and European Pediatric
and intuitive, as both the old and current literature indicate.
Neurology Society,4 – 6 or in published articles,7,8 on 40 additional
The performing musician is not necessarily creative at all, be-
patients presenting an easily recognizable pattern of protracted
cause playing or singing is primarily a kinesthetic, auditory, and
catastrophic encephalopathy unexpectedly followed by a relatively
repetitive motor task that relies on highly developed auditory favorable natural outcome.
and proprioceptive memory. Patients with anti-NMDAR encephalitis1 clearly fulfill all
This generates a number of testable hypotheses. criteria of the above-described encephalopathy.2,7,8,9 Future clini-
cal/biological correlations should further clarify whether anti-
The Alerion Institute, North Garden, VA NMDAR encephalitis and “Subacute Encephalopathy associated
with intense bursts of abnormal movements and long lasting cog-
nitive and behavioral disturbances” are unique —as supported by
Reference
a recent report9—or overlapping entities. Furthermore, the unrav-
1. Ann Neurol 2009;66:A7. DOI: 10.1002/ana.21890 eling of the likely autoimmune pathophysiological process is of
© 2010 American Neurological Association 141

ANNALS of Neurology
the greatest interest.10 However, whether anti-NMDAR is the pri- toms in anti-NMDAR encephalitis. This disorder, first reported
mary cause of the disease or just a very useful biomarker remains in 2007, is currently recognized worldwide due to the associa-
also to be elucidated. tion with a highly predictable syndrome and the availability of a
diagnostic test that we developed. The sharp increase of reports
suggests the disorder is frequent, but symptoms are still often
I thank the excellent colleagues who kindly reported to me, ascribed to other diagnoses. The Table lists the disorders con-
or in meetings, their observations: A. Arbor, S. Chabrier, sidered in our initial patients or in case reports of likely anti-
D. F. Clarke, T. Deonna, B. Echenne, S. Ghariani, M. NMDAR encephalitis. The use of descriptive terms brings an
King, S. M. Leber, A. Levy Gomes, M. C. Nassogne, A. D. analogy with the 7 blind men recognizing an elephant, where each
Rothner, E. Roulet, M. Tardieu, T. Temudo, and J. P. man identifies, at best, 1 part but not the whole of the elephant.
Vieira. Similarly, elements of the anti-NMDAR encephalitis syndrome
were previously described, but the entire picture and link to other
Neuropediatric Service, University of Sherbrooke, Sherbrooke, descriptions were missed. Relevant associations (eg, 50% of adults
Quebec, Canada have NMDAR-expressing tumors)1 were unnoticed, and empiric
management sometimes led to fatal consequences (Dalmau, un-
References
1. Florance NR, Davis RL, Lam C, et al. Anti-N-methyl-D-aspartate
Receptor (NMDAR) encephalitis in children and adolescents. Ann TABLE: Disorders Considered in Anti-NMDAR
Neurol 2009;66:11–18. Encephalitis
2. Sebire G, Devictor D, Huault G, et al. Coma associated with intense
bursts of abnormal movements and long-lasting cognitive Frequently Considered Diagnoses
disturbances: an acute encephalopathy of obscure origin. J Pediatr
1992;121:845– 851. Acute psychosis, schizophrenia, catatonia, malignant
3. Taylor T, ed. The devil came to St Louis! The true story of the catatonia
1949 exorcism. Decatur, IL: Whitechapel Productions, 2006. Infectious or postinfectious autoimmune
4. Clarke DF, Leber SM, Arbor A. The Sebire syndrome. Three encephalitis (viral, mycoplasma, PANDAS)
cases of a stereotyped encephalopathy with a good prognosis.
Ann Neurol 2000;48:S51. Drug abuse
5. Levy Gomes A. Coma associated with intense abnormal Neuroleptic malignant syndrome
movements: a new case. Eur J Paed Neurol 1999;3:A6.
Encephalitis lethargica
6. Ghariani S, Leber S, Clarke DF, et al. Acute encephalopathy with
long-lasting but reversible alteration of consciousness, dyskinesia, Hashimoto’s encephalopathy
and cognitive decline. Ann Neurol 2002;52:S147.
Descriptive Terms Used to Describe Disorders
7. Haimi-Cohen Y, Soen G, Amir J, et al. Coma with abnormal move-
ments and prolonged cognitive disturbances: a new subset of acute
Resembling Anti-NMDAR Encephalitis
encephalopathy. Neuropediatrics 1996;27:270 –272.
Demonic possession
8. Hartley LM, Ng SY, Dale RC, et al. Immune mediated chorea
encephalopathy syndrome in childhood. Dev Med Child Neurol Acquired reversible autistic syndrome in acute
2002;44:273–277. encephalopathic illness in children
9. Poconi C, Korff CH, Ricotti V, et al. Severe childhood encepha- Coma associated with intense bursts of abnormal
lopathy with dyskinesia and prolonged cognitive disturbance ev-
idence for anti-NMDAR encephalitis. Dev Med Child Neurol
movements and long-lasting cognitive and
2010. EPub ahead of print. behavioral disturbances
10. Tardieu M. Anti-NMDAR encephalitis: a new entity: Dev Med Immune-mediated chorea encephalopathy syndrome
Child Neurol 2010. EPub ahead of print. in childhood
DOI: 10.1002/ana.21928 Acute diffuse lymphocytic meningoencephalitisa
Acute reversible limbic encephalitisa
Reply Acute juvenile female nonherpetic encephalitisa
Nicole R. Florance, MD1 and Josep Dalmau, MD, PhD2
Juvenile acute nonherpetic encephalitisa
The suggestion that some patients with anti–N-methyl-D- AERRPSa
aspartate receptor (NMDAR) encephalitis may be regarded as a
Some patients initially categorized with these descriptive
possessed by the devil is not far fetched. As pointed out by Dr terms were subsequently proven to have anti-NMDAR
Sébire, many symptoms of anti-NMDAR encephalitis are in- encephalitis (reviewed by Iizuka et al4 and Dalmau
cluded in accounts of “demonic possession,” including “shouting [unpublished data]).
in unknown tongue.” Descriptions of the 14-year-old boy whose NMDAR ⫽ N-methyl-d-aspartate receptor; PANDAS ⫽
story inspired the book and film The Exorcist suggest that the pediatric autoimmune neuropsychiatric disorder associated
with streptococcal infections; AERRPS ⫽ acute encephalitis
boy was imperfectly repeating the Latin words of the priest. In- with refractory, repetitive partial seizures.
deed, echolalia and language disintegration are frequent symp-
142 Volume 67, No. 1

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LETTERS/REPLIES

Music and Mental Illness In Search of Lost Time

© 2010 American Neurological Association 141

142 Volume 67, No. 1

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