524 J Neurol Neurosurg 1998;64:524–528 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.64.4.524 on 1 April 1998. Downloaded from Akinetic mutism as a classification criterion for the diagnosis of Creutzfeldt-Jakob disease

Anke Otto, Inga Zerr, Maria Lantsch, Kati Weidehaas, Christian Riedemann, Sigrid Poser

Abstract damage among which are processes of Objectives—Among the classification cri- degeneration and circumscribed cerebral le- teria for the diagnosis of Creutzfeldt- sions, above all bilateral frontal and mesodien- Jakob disease, akinetic mutism is cephalic lesions. Several authors suggested described as a symptom which helps to that, when describing a complex symptomatol- establish the diagnosis as possible or ogy including dementia and a disturbance of probable. Akinetic mutism has been ana- consciousness, the diagnosis of “akinetic mut- tomically divided into two forms—the ism” should be waived and instead be replaced mesencephalic form and the frontal form. by the term “apallic syndrome”.2–3 Another The aim of this study was to delimit the approach is to use the term of akinetic mutism symptom of akinetic mutism in patients and to consider it as just one of several stages with Creutzfeldt-Jakob disease from the within a process leading to an apallic complex of symptoms of an apallic syn- syndrome.4 Akinetic mutism has been a solid drome and to assign it to the individual classification criterion for the diagnosis of pos- forms. sible and probable Creutzfeldt-Jakob disease Methods—Between April and December used by the European Creutzfeldt-Jakob dis- 1996, 25 akinetic and mute patients with ease surveillance unit since 1993. However, up Creutzfeldt-Jakob disease were consecu- to now no attempt has been made to explain tively examined. The patients were classi- the use of the term akinetic mutism for the fied according to the definition of akinetic diagnosis of Creutzfeldt-Jakob disease. To give mutism by Cairns and secondly in accord- a better understanding of this term and to show ance with the features constituting the the diVerences between akinetic mutism and complete picture of an appalic syndrome an apallic syndrome, the symptoms of the (defined by Gerstenbrand). patients with Creutzfeldt-Jakob disease were —From 25 patients with definite firstly classified according to the definition of Results 1 Creutzfeldt-Jakob disease, 24 patients akinetic mutism by Cairns et al and secondly showed impoverishment of speech and, in accordance with the features constituting the 5 after a mean duration of four (range complete picture of an apallic syndrome. 1.1–11.2) months, almost complete ab- sence of voluntary movements and Methods speech. Seven patients were classified as PATIENTS

being mute and akinetic and assigned to The group consisted of patients registered as http://jnnp.bmj.com/ the mesencephalic form whereas 13 pa- suspect cases of Creutzfeldt-Jakob disease at tients were classified as apallic. One the Department of Neurology and the Institute patient was mute without being akinetic of Neuropathology of the Georg-August- University, Göttingen. Since June 1993 206 and four patients were comatose. patients were visited in the notifying hospitals Conclusion—DiVuse brain damage un- Neurologische Klinik by a research physician and classified as prob- derlies akinetic mutism in patients with und Poliklinik, able or definite cases of Creutzfeldt-Jakob dis- Georg-August- Creutzfeldt-Jakob disease. The term can

ease. Among these patients, 110 were reported on October 2, 2021 by guest. Protected copyright. be used as a classification criterion for the Universität Göttingen, to be akinetic and mute at the time of notifica- Robert-Koch-Strasse diagnosis of Creutzfeldt-Jakob disease; tion to the study. Apart from medical history 40, 37075 Göttingen, however, it should be applied very care- and a physical examination, a standardised Germany fully and delimited clearly from the apal- A Otto epidemiological questionnaire was completed I Zerr lic syndrome. (J Neurol Neurosurg Psychiatry 1998;64:524–528) with the relatives of the patient containing M Lantsch questions about a detailed history. Further K Weidehaas C Riedemann Keywords: Creutzfeldt-Jakob disease; akinetic mutism; information was obtained from medical S Poser apallic syndrome records and medical staV. Twenty five patients were selected, who had been examined by one Correspondence to: of us personally and consecutively in the time Dr A Otto, Neurologische According to the definition of Cairns et al,1 aki- between April and December 1996. The medi- Klinik und Poliklinik, Georg-August-Universität netic mutism describes a condition of apparent cal history of these 25 patients provided Göttingen, Robert-Koch-Str. alertness along with a lack of almost all motor detailed data concerning the signs and symp- 40, 37075 Göttingen, functions including speech, gestures, and facial toms mentioned below describing the akinetic Germany. Telephone 0049 551 39 6636; fax 0049 551 expression. This definition is based on a case mutism and the apallic syndrome. 39 7020. description of a young female patient with an epidermoid cyst of the third ventricle. Later on, CLASSIFICATION CRITERIA FOR THE DIAGNOSIS OF Received 8 July 1997 and in the term of akinetic mutism was used in CREUTZFELDT-JAKOB DISEASE revised form 30 September 1997 connection with other comparable clinical pic- Patients were classified according to the crit- Accepted 3 October 1997 tures of diVerent aetiology and patterns of eria of Masters et al6 and neuropathological Akinetic mutism as a classification criterion for Creutzfeldt-Jakob disease 525 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.64.4.524 on 1 April 1998. Downloaded from

criteria.The diagnosis of Creutzfeldt-Jakob and into symptoms which are caused due to the disease is probable if the patient has had loss of functional systems inhibiting the brain rapidly progressive dementia during the past stem (symptoms 5–8). (1) Coma vigile, in two years, if the EEG of the patient shows peri- which the patient lies in bed with eyes open but odic sharp wave complexes, and if two of the staring into the distance, is neither able to fix following four additional symptoms appear: (1) the eyes on objects nor to follow them (by con- myoclonus; (2) visual or cerebellar symptoms; trast with the patient in a state of akinetic mut- (3) pyramidal or extrapyramidal signs; (4) aki- ism). The patient cannot show any adequate netic mutism. motor activity to external stimuli. (2) Distur- The diagnosis of Creutzfeldt-Jakob disease is bance of the normal sleep-waking cycle. (3) possible if the patient has had rapidly progres- Primitive patterns to sensory stimuli. External sive dementia during the past two years, if two stimuli cause a generalised motor reaction of all of the four above mentioned clinical signs are four limbs and the trunk, which clinically cor- seen, but the EEG is atypical. If the criteria are responds to mass movements. (4) Lack of not fulfilled another disease is presumed. For emotional reactions. (5) Abnormal position of the definite category the diagnosis was con- the limbs. In some cases a flexed position is firmed neuropathologically, immunocyto- seen, in other cases a stretched position of the chemically, and by prion positive western blots. legs is present. (6) Oculovestibular reflexes and reflexes of position. (7) Primitive motor 1 AKINETIC MUTISM DEFINED BY CAIRNS ET AL patterns. The primitive motor patterns can be The neurological status at the time of the divided into diVerent groups according to the physical examination was classified in accord- functional basis—for instance, the motor pat- ance with the first definition of akinetic mutism terns of the oral sense (sucking-chewing by Cairns et al.1 Apart from the above automatisms), the grasping reflexes, and the mentioned short description, Cairns et al1 mental reflexes (palmomental reflex). (8) delineated the condition of the patient in a state vegetative dysregulation of akinetic mutism as follows: “In the fully developed state he makes no sound and lies inert, except that his eyes regard the observer Results steadily, or follow the movement of objects, and Altogether 25 patients were included in the they may be diverted by sound. Despite his study; 18 women and seven men. The mean steady gaze, which seems to give promise of age at disease onset was 65.8 (SD 8.5); range speech, the patient is quite mute, or he answers 44–83) years. The mean time between appear- only in whispered monosyllables. Oft-repeated ance of the first symptoms and occurrence of commands may be carried out in a feeble, slow, akinetic mutism was four (range 1.1–11.2) and incomplete manner, but usually there are months. The mean duration of disease of all no movements of a voluntary character.” The patients was 7.5 (range 2-17) months. The most important features were extracted from time span between occurrence of akinetic mut- this description and summarised as follows— ism and date of death was a mean of 3.5 (range appearance of alertness—visual fixation of the 0.3–15) months. examining person—movements of the eyes in response to auditory stimuli— movements CLINICAL PRESENTATION AT THE ONSET AND IN http://jnnp.bmj.com/ after often repeated commands—speech THE COURSE OF THE DISEASE /speech eVort Seventeen patients (68%) showed signs of a rapidly progressive dementia syndrome at the SYMPTOMS WHICH, ACCORDING TO onset of the disease. Speech disorders with 5 GERSTENBRAND CONSTITUTE THE COMPLETE phonematic or semantic paraphasia, dysar- PICTURE OF AN APALLIC SYNDROME thria, perseverations, and impairments in The development of an apallic syndrome can speech understanding were seen in only five be caused in two diVerent ways7 : cases at the onset. Pseudobulbar speech was noticed in three cases at the beginning of the on October 2, 2021 by guest. Protected copyright. Type 1 disease, in two other cases it was noticed during Type 1 is a progressive degeneration of the cer- the subsequent observation period. Akinetic ebrum which can aVect the cortex and white mutism never appeared as an initial symptom. matter either separately or simultaneously. The In the course of the disease, all patients devel- apallic syndrome represents an irreparable end oped dementia and mutism which often mani- stage. fested itself in a reduction of spontaneous speech, construction of incomplete sentences, Type 2 and single word utterances. Type 2 is an acute process which leads to the 6 loss of the entire cerebral function. This may be CLASSIFICATION ACCORDING TO MASTERS et al caused by a diVuse damage to the cerebrum or According to the classification of Masters et al, by interruption of its connecting systems to the 22 patients were classified as probable and brain stem at various levels. The apallic three as possible cases (later confirmed as defi- syndrome can appear as an end stage or a tran- nite cases by neuropathological examination). sitory syndrome. The categories worked out by All patients had shown at least two of the four Gerstenbrand5 for the description of an apallic concomitant symptoms previously described syndrome can be divided into clinical symp- which are necessary to assume the potential toms which are caused by a lesion of structures diagnosis of Creutzfeldt-Jakob disease. In and systems of the cerebrum (symptoms 1–4) addition, six patients presented disturbances of 526 Otto, Zerr, Lantsch, et al J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.64.4.524 on 1 April 1998. Downloaded from

Table 1 Classification of the patients fulfilling the criteria syndrome were applied to the seven patients for an akinetic mutism syndrome (according to Cairns et al1) who were in the state of akinetic mutism (table 3). Many of these patients showed a disturbed n=24 n % sleep-waking cycle and a lack of emotions. One Appearance of alertness 7 28 patient responded with a smile, another looked Visual fixation of the examining person 7 28 Movements of the eyes in response to auditory 728 reluctantly and was moaning. Oculovestibular stimuli reflexes and a coma vigile could not be found. Movements after frequently repeated commands 3 12 All patients showed a visual fixation of the Spontanous speech/speech eVort 3 12 examining person. The symptoms of these patients thus diVer from those present in ocular motor control; two of them had a verti- patients with an apallic syndrome. It is striking cal gaze palsy. that abnormalities of posture and primitive motor patterns occurred twice as often in CLASSIFICATION OF THE PATIENTS ACCORDING TO patients with an apallic snydrome than in THE DEFINITION AND DESCRIPTION OF THE STATE patients with akinetic mutism. These symp- 1 OF AKINETIC MUTISM BY CAIRNS et al toms belong to the complex of symptoms 5–8 Seven of 25 patients fulfilled the criteria of aki- comprising disinhibited functions after a fail- netic mutism developed by Cairns et al1 . These ure of higher cortical systems. Proportionally, patients were awake, fixed their eyes on the vegetative dysregulation was found more often examining person, and reacted to auditory in patients who were in a state of akinetic mut- stimuli by moving their eyes (table 1). At the ism. At the time of investigation, four patients time of the examination one patient was mute had already been comatose—that is, their con- but able to walk and perform activities of daily sciousness was clouded and the sleep-waking living on his own and was therefore not cycle was disturbed.7 They are not included in assigned to a state of an akinetic mutism. In the tables 2 and 3. further progression of the disease he developed an akinetic mutism. Discussion CLINICAL PRESENTATION AT THE ONSET AND IN CLASSIFICATION OF THE PATIENTS ACCORDING TO THE COURSE OF THE DISEASE THE SYMPTOMS CONSTITUTING THE COMPLETE After a mean duration of four months, akinetic PICTURE OF AN APALLIC SYNDROME mutism occurred in 24 patients. However, 5 (GERSTENBRAND) mutism and akinesis must not develop simulta- Thirteen patients who presented a special kind neously. One patient was in a state of mutism of impaired consciousness in the sense of a and very apathic; however, he could walk and coma vigile were categorised into the complex was able to perform some activities of daily liv- of symptoms of an apallic syndrome. All these ing on his own. In accordance with the defini- patients showed an impaired sleep-waking tion of Cairns et al1 this patient, despite his cycle and no emotions of any kind. Table 2 apathy and , cannot be classified as shows the individual symptoms.The symptoms being akinetic and mute. In psychotic disorders constituting the complete picture of an apallic also, which originate from a loss of motivation

Table 2 Classification of the patients fulfilling the criteria for an apallic syndrome (according to Gerstenbrand et al5) http://jnnp.bmj.com/

Patients in the state of an apallic syndrome, n = 13 (group 2)

12345678910111213n%

1 Coma vigile xxxxxxxxxx x x x 13100 2 Disturbance of the normal xxxxxxxxxx x x x 13100 sleep-waking cycle 3 Primitive patterns to sensory stimuli x x x x x x x 7 54 4 Lack of emotional reactions xxxxxxxxxx x x x 13100 5 Abnormal position of the extremities x x x x x x x x x 9 69 on October 2, 2021 by guest. Protected copyright. 6 Oculovestibular reflexes and reflexes xx 215 of position 7 Primitive motor patterns x xxxx x x x 8 62 8 Vegetative dysregulation x x 2 15 Symptoms of the individual patients 5635654445 7 5 7 (n)

x = present.

Table 3 Application of the features of an apallic syndrome to patients in the state of akinetic mutism

Patients in the state of akinetic mutism, n=7(group1)

1234567n%

1 Coma vigile 2 Disturbance of the normal sleep-waking cycle x xxx457 3 Primitive patterns to sensory stimuli x x 2 29 4 Lack of emotional reactions xxxxx 571 5 Abnormal position of the limbs x x 2 29 6 Oculovestibular reflexes and reflexes of position 7 Primitive motor patterns x x 2 29 8 Vegetative dysregulation x x 2 29 Symptoms of the individual patients (n) 132434

x = present. Akinetic mutism as a classification criterion for Creutzfeldt-Jakob disease 527 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.64.4.524 on 1 April 1998. Downloaded from

but not from diVuse or circumscribed cerebral the clinical picture, into a hyperpathic and lesions, such as and , the somnolent form.8 The frontal form occurs in term should not be applied. In some of the patients with bilateral circulatory disturbances other 24 patients, who lay motionless in bed, in the supply area of the anterior cerebral motor activity could also be induced by artery.2 13–19 Other papers state that akinetic startling them through auditory stimuli. They mutism occurred in patients with glioblastoma often reacted with defensive reflexes of all four which had infiltrated bilaterally the rostral limbs and thus proved that, in a state of akinetic midline structures and medial parts of the mutism, they lie motionless but not paralysed .20 In various papers, examples for in bed. This aspect is also part of the definition mesodiencephalic lesions are given—for exam- of Cairns et al1 and Freemon.2 ple, in the case of a craniopharyngioma in the region of the third ventricle,10 21 damage to the 1 22 AKINETIC MUTISM ACCORDING TO CAIRNS et al and hypothalamus, a small mid- Cairns et al1 describe the symptomatology of brain lesion,23 lesions in the late stage of an their patient with a cyst in the third ventricle obstructive ,24 and circulatory with complete immobility, inability to speak, disturbances in the mesodiencephalic region.25 and showing adequate reactions to external These lesions always go along with perceptive stimuli. The condition of a patient with and cognitve deficiencies.3 Our patients would circulatory disturbances in the mesodien- have to be assigned to the mesencephalic type. cephalic region is characterised similarly by Segarra8 assigned two diVerent clinical appear- Segarra.8 The patient lay motionless but not ance forms to both anatomical localisations: paralysed in bed, was somnolent but not “hyperpathic” akinetic mutism to bilateral comatose, calm but not mute. The akinetic frontal lobe damage, “somnolent” or “apa- state of the patient of Cairns et al1 could imme- thetic” akinetic mutism to the mesencephalic diately be remedied by puncture of the cyst. region. On the one hand, both forms can be The child could speak normally and did not distinguished by the degree of vigilance and on show any other neurological deficiencies. At the other by the preservation of ocular motor the time of the disease, the child’s ability to control. Mesencephalic damage is typically move and speak seems to have been blocked. characterised by vertical gaze palsy and oph- There are similar reports from other authors, thalmoplegia. Interestingly, six patients from who noted an improvement in the symptoma- the selected group showed disorders of the tology of their patients.9–10 The case study of ocular motor control and two patients a vertical Lhermitte et al11 showed that some patients gaze palsy. Our patients would thus have to be who are in a state of akinetic mutism can even assigned to the mesencephalic type and som- be oriented relative to time and place and show nolent type of akinetic mutism. By contrast a certain understanding of speech. They could with the case descriptions mentioned above elaborate a communication code with a patient neuropathological findings in the brain of who had developed akinetic mutism after an patients with Creutzfeldt-Jakob disease are not ischaemic lesion of the reticular formation. By limited to the mesencephalon. On light micro- hand movements she indicated as to which of scopical examination, various degrees of the answers given in choice were correct and, spongiform change was observed in 189 for short periods during the day, she could patients examined by Brown .26 Lesions

et al http://jnnp.bmj.com/ answer with “yes” and “no”. By contrast with were typically seen in the neocortex, thalamus, this blockage of speech and motor activity basal ganglia, and cerebellum, but the com- caused by a circumscribed lesion, our patients parative severity varied greatly from patient to always developed dementia during a primary patient and within diVerent regions of the same degenerative process. According to brain. Therefore it seems unlikely that a corre- Kretschmer7 this means a quantitative reduc- lation will be found between neuropathological tion of cerebral performances, although cer- patterns and the akinetic mutism in patients ebral functioning is preserved to a certain with Creutzfeldt-Jakob disease. Nevertheless, on October 2, 2021 by guest. Protected copyright. degree. This process often also aVects speech no attempt has not been made, yet. and may entail a veritable degradation of speech. An attempt to develop a communica- APALLIC SYNDROME ACCORDING TO 5 tion code with the 25 patients examined, simi- GERSTENBRAND lar to the code elaborated by Lhermitte et al11 According to Kretschmer,7 the apallic syn- and his patient, was unsuccessful. There is a drome may represent the end stage of a chronic discrepancy, though, between speech produc- or acute process, such as a tumour disease or tion and speech understanding. One patient, in trauma respectively. Phases of a coma pro- a prestage to akinetic mutism, was mute but longé, parasomnia, and akinetic mutism may correctly followed simple instructions. This preceed a traumatic apallic syndrome which also occurred in some of the patients who were may turn into a coma during the course. The in a state of akinetic mutism. Lebrun defines phases of a prolonged coma and parasomnia mutism as a loss of speech while a clearly bet- occur after development of an acute ter speech understanding is preserved.12 This syndrome and are characterised by persistent definition is valid for patients with Creutzfeldt- coma and a somnolent state in which the eyes Jakob disease. Several years after its introduc- of the patient are closed. These phases do not tion by Cairns et al,1 the term akinetic mutism necessarily appear in patients with a degenera- was more exactly subdivided into a pathologi- tive process. However, the 25 patients showed cally anatomically localised frontal form and a diVerent stages. In the earliest stage of the dis- mesencephalic form and, in accordance with ease, one patient was mute but not akinetic, 528 Otto, Zerr, Lantsch, et al J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.64.4.524 on 1 April 1998. Downloaded from

seven were in a state of akinetic mutism, 13 in same.The main diVerences on clinical examin- a subsequent stage of an apallic syndrome, and ation are: patients with an apallic syndrome four patients in a final stage of a coma. In the (coma vigile) seem alert but without visual whole course of the disease, there was consid- fixation of objects and without evident acitivity erable time variation in reaching the individual of consciousness. By contrast, patients in a stages and the duration of the individual stages. state of akinetic mutism are alert with pre- The progression of the symptomatology is served visual fixation of objects. Although the reflected in the increase in symptoms appear- spontanous speech is lost, the activity of ing from the occurrence of akinetic mutism to consciousness may be evident through better the end stage of an apallic syndrome.Compara- speech understanding and the ability to carry tively, the symptoms describing a functional out simple commands. loss of higher cortical functions more often occur in patients with an apallic syndrome. 1 Cairns H, Oldfield RC, Pennybacker JB, et al. Akinetic mut- ism with an epidermoid cyst of the 3rd ventricle: with a This diVerence is much more obvious in the report on the associated disturbance of brain potentials. case of the symptoms characterising a disinhi- Brain 1941;64:273–90. bition of the autonomous functions. They 2 Freemon FR. Akinetic mutism and bilateral anterior cerebral artery occlusion. J Neurol Neurosurg Psychiatry occurred twice as often in patients with an 1971;34:693–8. apallic syndrome, except for the feature of a 3 Ackermann H, Ziegler H. Akinetischer Mutismus-eine Lit- eraturübersicht. Fortschr Neurol Psychiatr 1995;63:59–67. vegetative dysregulation which appeared as an 4 Avenarius HJ, Gerstenbrand F. Die Klinik des trauma- tischen apallischen Syndroms. In: Gerstenbrand F, ed. Das early symptom in both groups. If the descrip- . Wien: Springer, 1967:9. 7 traumatische apallische Syndrom tion of an apallic syndrome with the distur- 5 Gerstenbrand F. Das traumatische apallische Syndrom: Klinik, Morphologie, Pathophysiologie und Behandlung. Wien, New bance of the disinhibitory systems of the brain York: Springer, 1967:23–38. stem due to a blockage of the pallium is taken 6 Masters CL, Harris OA, Carleton Gajdusek D, et al. Creutzfeldt-Jakob disease: patterns of worldwide occur- as a basis for the division of the symptoms into rence and the singificance of familial and sporadic cluster- a complex of symptoms 1–4 and 5–8, the con- ing. Ann Neurol 1979;5:177–88. clusion is reached that decortication in patients 7 Kretschmer E. Das apallische Zeitschrift für diegesante Neurologie and Psychiatry 1940;169:567–79. with an apallic syndrome shows a more 8 Segarra JM. Cerebral vascular disease and behavior. 1. The advanced degree of development than patients syndrome of the mesencephalic artery (basilar artery bifur- cation). Arch Neurol 1970;22:408–18. in a state of akinetic mutism. Akinetic mutism 9 Grotjahn M. Klinik und Bedeutung akinetischer Zustände can therefore be considered as a prestage to an nach Luftfüllung des dritten Ventrikels. Monatsschrift für Neurologie und Psychiatrie 1936;93:121–39. apallic syndrome.The coma vigile is an impor- 10 Mason-Browne NL. Alteration of consciousness: tumor of the reticular activating system. Arch Neurol Psychiatry tant distinctive feature for the diagnosis of an 1956;76:380–7. apallic syndrome and an akinetic mutism. The 11 Lhermitte FC, Gautier R, Marteau R, et al. Troubles de la conscience et mutisme akinétique. Rev Neurol 1963;109: apallic patients, who are in a coma vigile, seem 115–31. to be alert with eyes open but do not respond to 12 Lebrun Y. Mutism. London: Whurr, 1990. any external stimulus. Formally, they have to 13 Nielsen JM, Jacobs LL. Bilateral lesions of the anterior cin- gulate gyri: report of case. Bull Los Angeles Neurol Soc 1951; be considered as being comatose. By contrast 16:231–4. with these patients, patients in a state of 14 Nielsen JM. Anterior cingulate gyrus and . Bulletin of the Los Angeles Neurological Society 1951;16:235– akinetic mutism are alert and able to contact 43. their surroundings, at least by eye contact. This 15 Faris AA. Limbic system infarction: a report of two cases. Neurology 1969;19:91–6. supports the impression of an at least partly 16 Buge A, Escourolle R, Rancurel G, et al. “Mutisme preserved activity of consciousness. On the akinétique” et ramolissement bicingulaire: trois observa- tions anatomo-cliniques. Rev Neurol (Paris) 1975;131:121– http://jnnp.bmj.com/ basis of the aforementioned diVerences be- 37. tween akinetic mutism and an apallic syn- 17 Kurtz D, Autret A, Ruchoux M. Mutisme akinétique soudain et anomalies. EEG chez une femme de 89 ans. Rev drome, the complex symptomatology in pa- Neurol (Paris) 1988;144:527–33. 18 Németh G, Hegedüs K, Molnár L. Akinetic mutism associ- tients with Creutzfeldt-Jakob disease should ated with bicingular lesions: clinicopathological and not be simply subsumed under the picture of functional anatomical correlates. Eur Arch Psychiatry an apallic syndrome. Patients with Creutzfeldt- Neurol Sci 1988;237:218–22. 19 Borggreve F, De Deyn PP, Mariën P, et al. Bilateral Jakob disease have to be assigned to the infarction in the anterior cerebral artery vascular territory mesencephalic form of akinetic mutism. They due to an unusual anomaly of the circle of Willis. Stroke 1994;25:1279–98. on October 2, 2021 by guest. Protected copyright. have to be distinguished from akinetic mutism 20 Lechi A, Pilleri G, Carreras M. Akinetic mutism due to caused by bifrontal brain damage, which glioma of the midline. Archiv für Psychiatrie und Nervenk- rankheiten 1975;220:1–7. creates a distinct syndrome without cognitive 21 Ross ED, Stewart RM. Akinetic mutism from hypothalamic deficiencies or other neurological deficiencies. damage: successful treatment with . Neurology 1981;31:1435–9. The term akinetic mutism has been used as a 22 Shinoda M, Tsugu A, Oda S, et al. Develoment of akinetic classification criterion for the diagnosis of mutism and hyperphagia after left thalamic and right hypothalamic lesions. Childs Nerv Syst 1993;9:243–5. Creutzfeldt-Jakob disease, although the term 23 Williams D. Cerebral basis of temperament and personality. apallic syndrome would have been more Lancet 1954;ii:1–4. 24 Moser A, Freyberger HJ, Bruckmann H, et al. Akinetischer appropriate for many instances. Many patients Mutismus bei dekompensiertem triventrikuleren Hydro- zephalus. Fortschr Neurol Psychiatr 1995;63:248–51. originally classified as akinetic and mute were 25 Hochman MS, Sowers JJ, Bruce-Gregorios J. Syndrome of probably already apallic. For practical reasons the mesencephalic artery: report of a case with CT and necropsy findings. J Neurol Neurosurg Psychiatry 1985;48: it is not useful to change the classification 1179–81. criteria. Therefore the term akinetic mutism 26 Brown P, Gibbs Jr CJ, Rodgers-Johnson P, et al. 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