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patient underwent surgery. Because of dystonia after pallidal stimulation or palli- LETTERS thoracic kyphosis, he could not be placed dotomy. in a head coil or neck quad. We therefore In our opinion, compared with pallido- obtained a pre-operative CT scan for ‘‘indivi- tomies in Parkinson’s disease, the target for Long term results of unilateral dual targeting’’. The coordinates of a target in TD and dystonia could be anatomically posteroventral pallidotomy for the posteroventral globus pallidus (GP) were slightly different—that is, more lateral in established (2 mm in front of the mid the posteroventral GP perhaps because of antipsychotic drug induced anterior–posterior commissure (AC-PC), different activity of the GpE. More study into tardive dyskinesia 5 mm below the AC-PC and 21 mm lateral this pathology is needed. Tardive dyskinesia (TD) is a well known side of the AC-PC (head width 16 cm, third effect of neuroleptic drug treatment, and may ventricle width 6 mm, AC-PC 25 mm long)). M W P M Lenders, H P J Buschman coexist with tardive dystonia.1 It can be Five lesions were made at 8, 6, 4, and 2 mm, Department of Neurosurgery, Medical Spectrum Twente, Enschede, The Netherlands treated with several drugs, although they and on target at 82˚for 60 s. As this did not result in a satisfying improvement of dyski- rarely lead to a complete removal of symp- M D I Vergouwen toms. The main treatment of TD consists of nesia, a 2.0 mm diameter unipolar radio- frequency electrode (Fisher TCU 003) was Department of Neurology, Academic Medical Center, gradual neuroleptic drug dose reduction and Amsterdam, the Netherlands where possible complete withdrawal.2 inserted 2 mm lateral to the first target point. Nevertheless, in 40% of TD cases, symptoms A second series of lesions was performed as E N H J Steur do not disappear within 5 years of drug above, which resulted in a complete disap- Department of Neurology and Movement Disorder withdrawal. Wang et al first described a pearance of abnormal movements. Unit, Medical Spectrum Twente, Enschede, The pallidotomy as a treatment modality for Post-operatively, no complications were Netherlands TD.3 In severe cases of TD and dystonia, this observed. Involuntary grumbling was can lead to significant amelioration of abnor- reduced by 95%. The patient was discharged PKo¨lling mal movements and pronounced improve- from hospital 7 days after the operation, after Department of Psychiatry, Medical Spectrum Twente, Enschede, The Netherlands ment in function and quality of life.4 withdrawal of phenytoin, dexamethasone, and ciprofloxacin. The post operative UDRS M Hariz score was 2, and dyskenesia measured with PATIENT AND METHODS the UPDRS was 0. Unit of Functional Neurosurgery, Institute of Neurology, Queens Square, London, UK We report a 51 year old, chronically hospita- A post-operative CT scan of the brain lised man with a 29 year history of schizo- showed a lesion centred 23 mm lateral of Correspondence to: Dr H P J Buschman, Department phrenia. During this period, he was treated the midline in the calculated target point in of Neurosurgery, Medisch Spectrum Twente, PO Box

successfully for his psychotic symptoms with the left GPi. One year after this pallidotomy, 50 000, 7500 KA Enschede, The Netherlands; copyright. depot haloperidol and cisordinol. In 1993, he the patient was functioning very well. He was [email protected] developed TD. Initially it consisted of an very content with the nearly complete aboli- involuntary tic (myoclonus-like movements tion of TD, which allowed him again to paint, doi: 10.1136/jnnp.2004.044438 of the orofacial muscles), referred to in the travel, and visit his family. Now, 5 years after literature as tardive tic.5 Both hands showed the operation, he is still functioning very well, Competing interests: none declared dyskinetic and myoclonic movements, the with no signs of recurrence of dyskinesia and left hand less pronounced than the right, and dystonia. His UDRS and UPDRS scores there was severe acathisia. Haloperidol treat- remain at 2 and 0 respectively. REFERENCES ment was discontinued and symptoms of 1 Burke RE, Fahn S, Jankovic J, et al. Tardive psychosis relapsed. Other tried dystonia: late-onset and persistent dystonia included (6 mg/day), sulpiride DISCUSSION caused by antipsychotic drugs. Neurology (400 mg/day), (200 mg/day), In this case report, we describe the outcome 1982;32:1335–46. 2 Gerlach J, Casey DE. Tardive dyskinesia. Acta

dexetimide (1.5 mg/day), oxazepam (50 mg/ of posteroventral pallidotomy in a patient http://jnnp.bmj.com/ day), and clonazepam (1.5 mg/day). with drug induced TD. As more experience is Psychiatr Scand 1988:369–78. Treatment with (up to 600 mg/ gained,34 posteroventral pallidotomy, a pro- 3 Wang Y, Turnbull I, Calne S, et al. Pallidotomy for cedure with documented effects on parkin- tardive dyskinesia. Lancet 1997;349:777–8. day) and valproate (600 mg/day) improved 4 Weetman J, Anderson IM. Bilateral the psychosis and the TD temporarily, but sonian dyskinesias and dystonia, seems to be posteroventral pallidotomy for severe symptoms returned and became severely an effective treatment to improve or abolish antipsychotic induced tardive dyskinesia and devastating and invalidating, because TD symptoms of this movement disorder. The dystonia. J Neurol Neurosurg Psychiatry progressed to involve choreo-athetotic move- effect is not only apparent immediately and 1997;63:554–6. ments, most pronounced on the right side of during the first months after pallidotomy, but 5 Kurlan R, Kersun J, Behr J, et al. Carbamazipine the body and the midline. The patient also as is shown in this patient can be maintained induced tics. Clin Neuropharmacol developed choreo-athethotic movements of for long period of time. 1989;12:298–302. on September 28, 2021 by guest. Protected the jaws, tongue, lips, neck, and upper chest Gpi target localisation was based on CT as muscles, leading to improper and irregular an MRI was anatomically not possible. Ths breathing, involuntary grumbling, production would have been a good case for microelec- of uncontrollable noises, and frequent peri- trode recording, which at the time was not An Iranian family with congenital ods of pain in the chest. There was dysarthria, available at our hospital. Instead, we overlaid myasthenic syndrome caused by but no abnormal swallowing. He could walk, two CT images, taken parallel to the AC-PC a novel receptor but only in a very dyskinetic manner. Socially line to enhance contrast, and compared them he was totally isolated. with the concurrent Schaltenbrand atlas mutation (CHRNE K171X) Huntington’s disease and Wilson’s disease page. This indicated that in the second, (AChR) deficiency is were excluded. The pre-operative Unified effective, series of lesions we targeted the the most common form of congenital Dystonia Rating Scale (UDRS) Revised score Gpi close to the border of the Gpe. In myasthenic syndrome (CMS). Most AChR was 24. Dyskenesia and dystonia, as scored Parkinson’s disease, very often a very small deficiencies are caused by mutations in the by the Unified Parkinsons Disease Rating lesion in the Gpi can immediately be very coding region of the AChR epsilon subunit.1 Scale (UPDRS) were rated as 4 for both. successful; however in this our patient such a We report an Iranian Muslim family from the Owing to the unsuccessful medical treat- ‘‘parkinson GpI lesion’’ had no immediate province of Eastern Azerbaijan (Maragheh) ment, and onset of a rapid deterioration in visible effect. This does not exclude the in which three of five offspring of consangui- dyskinetic symptoms in the right side of possibility that over a longer period of time neous parents had early onset CMS arising the body, we decided on left sided poster- positive postoperative results could have from a newly described mutation in the oventral pallidotomy. In July 1999, the evolved, as is often observed in patients with epsilon subunit of the AChR; this mutation

www.jnnp.com 1040 PostScript J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.058149 on 16 June 2005. Downloaded from has been identified homozygously in all the frequency repetitive nerve stimulation. Anti- of other patients, including Europeans, with three sibs. This is the first report of an AChR AChR antibodies were negative in all affected AChR epsilon mutations.1 Although congeni- epsilon subunit mutation in Iran. sibs. tal joint contractures have not been pre- viously reported in CMS patients with AChR epsilon mutations, one of our cases (case 3) Case presentation Genetic analysis had mild flexion contracture of her left knee, The affected brother (case 1) was 23 years old All 12 exons, adjacent intronic regions, and although, in contrast to patients with RAPSN and the affected sisters were 19 (case 2) and the promoter region of the AChR epsilon mutations, none of our cases had arthrogry- 16 (case 3) years old. The unaffected brother subunit gene (CHRNE, GenBank accession posis multiplex congenita.15A distinguishing was 21 and the healthy sister was 13 years number AF105999/gi4580858) were ampli- feature in cases 1 and 3 was the presence of old. There was no history of miscarriage or fied by PCR. PCR amplified fragments were asymmetric elbow hyperlaxity. Joint laxity infant mortality in the family, although, purified with the NucleoSpin Extract kit has not been previously reported in any type according to verbal accounts from the (Macherey-Nagel, Du¨ren, Germany) and of CMS; however, it can be seen in some mother, fetal movements were decreased in sequenced with an Applied Biosystems model congenital myopathies. We postulate that all the affected sibs and case 1 had a difficult 3100 Avant DNA sequencer and fluorescence fetal hypotonia in the presence of intrauter- labour. The clinical diagnosis of CMS in this labelled dideoxy terminators (Perkin-Elmer, ine biomechanical forces might have influ- family had been made first in 1996. All cases Foster City, CA, USA). enced the normal modelling of the elbow presented with neonatal hypotonia, regurgi- Screening for mutation K171X of the joint. tation, ptosis (case 1 developed ptosis at the CHRNE gene was performed by restriction It seems that the incidence of CMS in Iran age of 6 months), and delayed motor mile- digest of PCR products in the patients and the is similar to that in other countries. Recogni- stones. The course of the disease had been healthy siblings. A 304 bp fragment contain- tion of differing features of CMS could help slowly progressive, transiently exacerbated by ing exon 6 was amplified by PCR from establish a definite genetic diagnosis and stress. Generalised weakness was more genomic DNA using primers 59- help implement appropriate measures. severe in the afternoons. Treatment with AGGTACAGATGGGAACAGAG-39 and 59- prednisone for 1 year yielded no improve- TCTGGACCCCGTCTAGAAGCG-39.ABfaI ment. There was positive response to pyr- digest yields 218, 71, and 15 bp fragments Acknowledgements idostigmine bromide (Mestinon) and the for the wild type allele. The mutation K171X We thank the patients and their family for their required doses had gradually been increased. introduces a new BfaI restriction site, there- kind participation in this study. There were no other affected relatives in the fore resulting in fragments of 153, 71, 65, and family. 15 bp in length (fig 1). P Soltanzadeh On clinical examination, all patients were Analysis of the CHRNE gene revealed a Department of Neurology, Tehran University of ambulant, although with limited walking homozygous nonsense mutation K171X Medical Sciences, Tehran, Iran distance. There was bilateral ptosis and (511ART) in all three affected siblings. The JSMu¨ller limited eye movements. The bulbar muscles two healthy siblings did not carry this Molecular Myology Laboratory, Friedrich-Baur- were also involved, manifesting as nasal mutation. DNA of the parents was not available for genetic analysis. The mutation Institute, Department of Neurology, Ludwig- speech (more severe in case 1), chewing Maximilians-University, Munich, Germany problems (in cases 1 and 3), swallowing causes a premature translation stop in exon 6 difficulties, and bilateral facial weakness. of the epsilon subunit of the AChR. Position A Ghorbani Pupillary response to light was normal. 171 is located in the N-terminal extracellular Department of Neurology, Tehran University of copyright. Muscle involvement was predominantly domain of the epsilon subunit protein. The Medical Sciences, Tehran, Iran proximal. Cases 1 and 3 had a waddling gait, mutation has not been previously described especially in the afternoons. Deep tendon in the literature. A Abicht, H Lochmu¨ller reflexes were normal. There was no muscle Molecular Myology Laboratory, Friedrich-Baur- wasting, scapulae alatae, scoliosis, or respira- Institute, Department of Neurology, Ludwig- Discussion Maximilians-University, Munich, Germany tory distress. No hospitalisations for respira- Severe endplate AChR deficiency can result tory problems were reported. Case 1 had left from different types of recessive mutations in A Soltanzadeh elbow hyperlaxity and case 3 also revealed the AChR subunit genes. As the mutation Department of Neurology, Tehran University of severe bilateral elbow hyperlaxity (more identified in this family lies in the N-terminal Medical Sciences, Tehran, Iran severe on the left side). Case 3 had mild region of the epsilon subunit, a putative flexion contracture of her left knee. translation product would not be inserted Correspondence to: Dr Payam Soltanzadeh, Muscle enzymes were normal. into the membrane and expressed at the cell Department of Neurology, Shariati University Electromyography of nasalis muscles showed Hospital, Kargar-Shomali Avenue, Tehran 14114,

surface. Another possibility is the degrada- Iran; [email protected] http://jnnp.bmj.com/ decrements (60%, 53%, and 35% in cases 1, 2, tion of the mutated epsilon subunit mRNA and 3, respectively) in response to low containing a premature stop codon in exon 6 doi: 10.1136/jnnp.2004.059436 by nonsense mediated decay. Therefore, we hypothesise that the K171X mutation leads to This work was supported by grants from the Deutsche a deficiency of AChR at the endplate. Forschungsgemeinschaft (DFG) to HL and AA. JSM Mutations in the coding region of the receives a scholarship from the Boehringer Ingelheim AChR epsilon subunit are said to be a Fonds. common cause of CMS in eastern Competing interests: none declared II:1 II:2 II:3 II:4 II:5 Mediterranean countries.2 Most of the CHRNE mutations reported so far are null on September 28, 2021 by guest. Protected bp bp mutations leading to receptor deficiency at References 218 200 the endplate. However, cases of homozygous 1 Burke G, Cossins J, Maxwell S, et al. Distinct nonsense mutations, as reported in our case, phenotypes of congenital acetylcholine receptor 153 are rather rare compared to the number of deficiency. Neuromuscul Disord 2004;14:356–64. 100 frameshift and missense mutations. AChR 2 Middleton L, Ohno K, Christodoulou K, et al. 80 71 epsilon subunit mutations have not yet been reported from Iran, despite the high rate of Chromosome 17p-linked myasthenias stem from 65 defects in the acetylcholine receptor epsilon- consanguineous marriages in the country. subunit gene. Neurology 1999;53:1076–82. CMS associated with facial malformations 3 Goldhammer Y, Blatt I, Sadeh M, et al. jn59436.f1 3 has been reported in Iranian and Iraqi Jews, Congenital myasthenia associated with facial who were subsequently found to have a malformations in Iraqi and Iranian Jews: a new Figure 1 Pedigree and restriction enzyme homozygous mutation (–38ARG) in an E- genetic syndrome. Brain 1990;113:1291–306. analysis of the Iranian CMS family. The affected box element within the promoter region of 4 Ohno K, Sadeh M, Blatt I, et al. E-box mutations siblings (II:1, II:3, and II:4) are homozygous for the RAPSN gene.4 Another similar patient of in the RAPSN promoter region in eight cases with the mutation CHRNE K171X (511ART); the Iranian Jewish origin has been reported with congenital myasthenic syndrome. Hum Mol Genet 2003;12:739–48. unaffected siblings (II:2 and II:5) do not carry the same founder mutation in a series of 1 5 Mu¨ller JS, Abicht A, Christen HJ, et al. A newly the mutation. The mutation CHRNE K171X early onset CMS cases. identified chromosomal microdeletion of the creates a new BfaI site. DNA of the parents was The clinical features of our cases with rapsyn gene causes a congenital myasthenic not available for genetic studies. CHRNE K171X mutation are similar to those syndrome. Neuromuscul Disord 2004;14:744–9.

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Transient ischaemic attack with References trigeminal autonomic symptoms 1 Goadsby PJ, Lipton RB. A review of paroxysmal Trigeminal autonomic cephalalgias present hemicranias, SUNCT syndrome and other short- with excruciating headaches and accompany- lasting headaches with autonomic feature, including new cases. Brain 1997;120:193–209. ing autonomic features such as ipsilateral 2 Davey R, Al-Din A. Secondary trigeminal lachrymation, rhinorrhoea, and eyelid ptosis. autonomic cephalalgia associated with multiple For clinical purposes, these headaches are sclerosis. Cephalalgia 2004;24:605–7. subclassified into cluster headaches, parox- 3 Galende AV, Camacho A, Gomez-Escalonilla C, ysmal hemicranias, and the SUNCT syn- et al. Lateral medullary infarction secondary to drome (sudden unilateral neuralgiform vertebral artery dissection presenting as a headache with conjunctival injection and trigeminal autonomic cephalalgia. Headache tearing),1 of which the paroxysmal hemicra- 2004;44:70–4. nia subtypes such as the acute and chronic 4 van Vliet JA, Ferrari MD, Haan J, et al. Trigeminal autonomic cephalalgia-tic-like hemicranias are indomethacin responsive. syndrome associated with a pontine tumour in a Neuroimaging is often normal in trigeminal one-year-old girl. J Neurol Neurosurg Psychiatry autonomic cephalalgias; nevertheless MRI 2003;74:391–2. should be considered, as there are associa- 5 Giffin NJ, Goadsby PJ. Basilar artery aneurysm tions between trigeminal autonomic cepha- with autonomic features: an interesting lalgia and multiple sclerosis,2 lateral pathophysiological problem. J Neurol Neurosurg medullary infarction,3 pontine tumours,4 Psychiatry 2001;71:805–8. basilar aneurysms,5 and other posterior fossa lesions. We present an unusual case of Tympanic measurement of body recurrent transient ischaemic attacks (TIA) with symptoms reminiscent of a trigeminal Figure 2 T2 Weighted magnetic resonance temperature in stroke patients autonomic cephalalgia which ceased after a image showing an acute left ponto- ‘‘turned on its ear’’ mesencephalic infarct. ponto-mesencephalic infarct. Body temperature is an important prognostic factor in acute stroke, and it is a marker of Case report (one week after the onset of symptoms) life threatening infections such as pneumo- nia.1 In clinical practice rectal temperature is A 65 year old women was admitted with a showed an acute infarct at the left ponto- regarded as a valid and reliable indicator of three day history of stereotyped neurological mesencephalic junction (fig 2). At follow up body temperature, but in the past 15 years episodes. These were characterised by a 10 days later, her gait had improved. Her tympanic infrared thermometers have been burning sensation in the left side of face, episodic symptoms had disappeared. introduced into widespread clinical use. congestion of the left eye, lachrymation from Tympanic temperature measurement is the left eye, watery discharge from the left easier, faster, and less invasive than rectal nostril, slurring of speech, and difficulty in Comment measurement. However, concerns remain walking. These episodes were not accompa- Cranio-facial autonomic symptoms such as ipsilateral lachrymation, rhinorrhoea, conjuc- about the reliability and validity of this nied by headache. Each episode lasted for copyright. method.2 around 30 minutes and occurred three or tival congestion, ptosis, or eyelid oedema are four times a day. On examination, there were the sine qua non of trigeminal autonomic The product manual of the tympanic no focal neurological deficits. In hospital, she cephalalgia. It is thought that activation of thermometer warns against overestimation had four more episodes. As she had a left the trigeminal afferent system generates of body temperature when the patient has mature cataract (fig 1), the possibility of pain, and co-activation of the VIIth nerve been lying on one ear. Many stroke patients glaucoma induced by lens rupture was efferent parasympathetic pathway produces are hemiparalytic or hemiparetic and there- considered and ruled out. Computed tomo- the autonomic manifestations such as ipsi- fore may lie on one ear. The aim of our study graphy of the brain was unremarkable. The lateral lachrymation and rhinorrhoea. A was to investigate the error in tympanic possibilities considered were a vertebro-basi- mechanism analogous to this can be evoked temperature measurements in patients who lar TIA and a trigeminal autonomic cepha- to explain our patient’s symptoms. She had have had a stroke and have been lying on one lalgia. As she had additional neurological an ischaemic stroke in the ponto-mesence- ear in this way. symptoms, a TIA was considered more likely phalic junction. Ischaemia in this region For this observational study we included could involve the trigeminal main sensory patients who had had an ischaemic or than trigeminal autonomic cephalalgia, and http://jnnp.bmj.com/ she was started on aspirin and intravenous and mesencephalic nuclei, resulting in facial haemorrhagic stroke and were admitted to heparin. Indomethacin was begun at 75 mg/ dysaesthesia comparable to ischaemic nerve the stroke unit of a university medical centre day but had to be discontinued after a single pain. In contrast to the ‘‘boring’’ intense pain or to the stroke rehabilitation unit of an dose because of gastric distress. Two days often encountered with trigeminal autonomic affiliated nursing home, both in an urban later, her episodic symptoms disappeared; cephalalgias, the burning dysaesthesias are area. Exclusion criteria were inability to lie on however, she now complained of persistent more consistent with ischaemia. Ischaemia one ear and absence of a rectal cavity. We could also trigger trigemino-facial synapses used the NIH stroke scale (NIHSS) to assess right sided weakness and difficulty in walk- 3 ing. On examination, she had a new left sided in the superior salivatory nucleus and switch stroke severity. No follow up was conducted. Horner’s syndrome, right upper motor neu- on the facial efferent parasympathetic path- Tympanic temperature was measured in on September 28, 2021 by guest. Protected rone facial palsy, and right upper limb way, resulting in ipsilateral lachrymation and both ears after the patient had been lying on weakness. Deep tendon reflexes were brisker rhinorrhoea. one ear of choice for at least 15 minutes (first on the right than on the left. Magnetic To the best of our knowledge, this is the measurement). The measurements were car- resonance imaging of the brain at this time first report of a TIA presenting with trigem- ried out in patients on waking in the morning inal sensory-autonomic symptoms. This or after an afternoon nap. The ear the patient report highlights the expanding spectrum of had been lying on and the other ear will be trigeminal autonomic cephalalgias and further referred to as the lower ear and the emphasises the need to rule out a vertebro- upper ear, respectively. The second measure- basilar TIA in elderly patients with a new ment was conducted by a different investi- gator—who was not aware of the results of onset of trigeminal autonomic cephalalgia, the first measurement—approximately 10 especially if additional neurological symp- minutes after the patient had no longer been toms are present. lying on one ear, and consisted of tympanic B V Maramattom and rectal temperature measurements. Department of Neurology, Lourdes Hospital, Kochi, To assess reliability we computed the South India; [email protected] difference between lower and upper ear temperature in consecutive tympanic mea- Figure 1 Congestion of the left eye during an doi: 10.1136/jnnp.2004.061366 surements and analysed this difference gra- acute attack. The patient gave written consent phically, as described by Bland and Altman.4 for this photograph to be reproduced. Competing interests: none declared Validity was assessed by comparing the mean

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investigations and treatment with antibiotics psychiatrists, and neuropsychologists. Brain in a considerable number of patients; more- Fiction by William Hirstein is the first book to over, an error of this size could have place confabulation at the centre of its 2 decreased the statistical power of clinical attention. By doing so, Hirstein is faced with the challenge of presenting and examining 1.5 trials of temperature lowering treatment in acute stroke patients, if tympanic tempera- the various discussions surrounding the 1 ture was used without attention being paid to definition, the subtypes, the neural, and 0.5 the side the patient was lying on.5 cognitive basis of confabulation and crucially the relation between its various forms and 0 manifestations. Brain Fiction, however, has –0.5 Acknowledgements taken up further challenges. By borrowing The support of the nursing staff of the stroke units and integrating data and notions from both (lower ear – upper ear) –1 of the Erasmus Medical Centre (Rene´Rook) and 36.5 37 37.5 38 38.5 neuroscience and epistemology, Hirstein puts nursing home Antonius Binnenweg (Hannie van forward an original definition and model of Average tympanic temperature Gijsen) is gratefully acknowledged. Difference in tympanic temperature confabulation, as a dynamic interplay of ((lower ear + upper ear)/2) A J J Rampen, E J van Breda, D W J Dippel creative and ‘‘checking’’ mental processes. More generally, Hirstein chooses confabula- Figure 1 Difference in tympanic temperature Department of Neurology, Erasmus Medical Centre, Rotterdam, Netherlands tion as a promising template for the formula- taken from the upper and lower ear, plotted tion of an interdisciplinary dialogue and against the average tympanic temperature Correspondence to: Dr Eric J van Breda, Department interchange of ideas between neuroscience, taken from both ears (Bland–Altman plot). The of Neurology, Erasmus Medical Centre, PO box psychology, and philosophy. In addition, the solid horizontal line in the centre indicates the 1738, 3000 DR Rotterdam, Netherlands; http:// book proposes to hold a place for confabula- mean difference between the tympanic www.pais-study.org/ tion in a continuum of behaviours, ranging temperatures (0.39˚C), while the dashed from ‘‘normal’’ other, and self-deception doi: 10.1136/jnnp.2004.058149 horizontal line indicates no difference (the null attempts in everyday life to deficits of theory hypothesis). Competing interests: none declared of mind, awareness, and symptoms of socio- pathy. tympanic temperatures with mean rectal References It should be evident from the above, that temperature. Brain Fiction is addressed to professionals of We studied 30 patients (nine male, 21 1 Reith J, Jorgensen HS, Pedersen PM, et al. Body diverse fields and Hirstein has tried to female). Their mean age was 69.5 years, and temperature in acute stroke: relation to stroke accommodate the potential clefts in acquain- severity, infarct size, mortality, and outcome. tance with expert knowledge and technical their median NIHSS score was 11, ranging Lancet 1996;347:422–5. from 0 to 38. The mean of the first tympanic 2 Varney SM, Manthey DE, Culpepper VE, et al. A terms. However, the specialised reader should temperature taken from the lower ear was comparison of oral, tympanic, and rectal keep in mind that the book does not offer an 37.6˚C. The mean tympanic temperatures temperature measurement in the elderly. J Emerg examination exhaustive in content or ency- taken from the upper ear were both 37.2˚C Med 2002;22:153–7. clopaedic in format. The book is of limited and the second measurement from the lower 3 Brott T, Adams HP, Olinger CP, et al. interest to clinicians. It mainly aims at ear had a mean of 37.3˚C. Mean rectal Measurements of acute cerebral infarction: a disentangling confabulation from the strict temperature was also 37.3 C. The mean clinical examination scale. Stroke boundaries of its hitherto neuroscientific ˚ copyright. 1989;20:864–70. examination and exposing it to direct philo- difference between the two ears was 0.39˚C 4 Bland JM, Altman DG. Statistical methods for (95% confidence interval, 0.22 to 0.56). This assessing agreement between two methods of sophical enquiry. This is an endeavour that difference ranged from 20.4˚C to 1.7˚C (fig 1). clinical measurement. Lancet, 1986;i, 307–10. promises mutual interdisciplinary benefits. The mean difference between the first mea- 5 van Breda EJ, van der Worp HB, van Gemert HM, Yet the author, perhaps motivated by the surement taken from the lower ear and the et al. Behandeling van een beroerte door existing lack of theoretical and descriptive rectal temperature was 0.29˚C (0.13 to 0.45). verlaging van lichaamstemperatuur; consensus on the subject, also chooses to ‘‘Paracetamol (acetaminophen) in stroke’’ (PAIS): propose a new aetiological account of the start van een klinische trial. Ned Tijdschr phenomenon in neuroscientific terms. Comment Geneeskd 2003;147:1976–8. Inevitably, this analysis often entails smooth- Our study showed a clinically significant ing of the hard edges of some conflicting difference between tympanic temperature neuroscientific findings, and partial coverage measurements in the two ears after a stroke of some complex issues raised by confabula- patient had been lying on one ear. This BOOK REVIEW tion, such as its implications for theories of difference disappeared after a while when consciousness, self-formation, and motiva- http://jnnp.bmj.com/ the patient was no longer lying on one ear. Brain fiction, self-deception and the tion. Such selectivity though has noteworthy There was no relation between actual body benefits. The book introduces an unprece- temperature and the size of the measurement riddle of confabulation dented emphasis in the study of confabula- error. Although the manufacturer of the tion by placing the definition, taxonomy, and tympanic thermometer cautions against Edited by William Hirstein. Published by Wiley, implications of the phenomenon into episte- heating of the auditory canal when the 2004, £22.95 (hardcover), pp 288. ISBN mological perspective. Thus, it sets the patient has been lying on one ear, especially 0262083388. ground for fruitful neuro-philosophical dis- in children, neither this effect nor its magni- cussions and it refreshes the way neuro- tude is well known. The striking neuropsychological symptom of logists, psychiatrists, and psychologists view on September 28, 2021 by guest. Protected In our opinion, a systematic error of 0.4˚C confabulation represents a prototypical form this and other related symptoms. on average is not acceptable from a clinical of false remembering and as such has point of view. It may lead to unnecessary stimulated great interest among neurologists, A Fotopoulou

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