Akinetic Mutism As a Classification Criterion for the Diagnosis Of
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524 J Neurol Neurosurg Psychiatry 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 brain 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.