Verbal perseveration as the initial symptom in a case of Creutzfeldt‐Jakob disease

The authors would like to thank Karin Geisler, Maura Bayer, and Caroline Brew for their critical reading of this paper, and the colleagues who provided the two reviews of this manuscript for their constructive comments and suggestions.

Abstract

Background: In the literature on cortical degenerative processes and primary progressive aphasia, language disorders have been discussed as the initial sign of higher cortical dysfunction. Verbal perseveration has also been cited to precede the occurrence of other cortical signs. In this paper we present language data from an autopsy‐confirmed case of Creutzfeldt‐Jakob disease (CJD) who exhibited profound verbal perseveration as the initial symptom.

The authors would like to thank Karin Geisler, Maura Bayer, and Caroline Brew for their critical reading of this paper, and the colleagues who provided the two reviews of this manuscript for their constructive comments and suggestions.

Aims: This case report aims to provide a description of the extent and pattern of verbal perseveration in a client with the rapidly progressing fatal CJD. Language test data and medical data, i.e., EEG and neuropathology—which confirm the diagnosis of CJD—are presented.

Methods & Procedures: Language testing was performed with our patient to determine the nature of the language disorder at a time when the diagnosis of CJD had not yet been made. The tape‐recorded sessions were transcribed and the language data analysed.

Outcomes & Results: The pervasive perseverative behaviour mirrors the underlying language‐processing difficulties and the magnitude of the progression of the disease process. Perseverative errors account for between 87% and 95% of the language‐processing errors on the various language tasks. In general, the first items of each task were responded to correctly, indicating that our client was trying to meet the various test demands and at the time of testing was still able to process language, albeit in a very restricted manner.

Conclusions: The rapid deterioration of language functions in our case and in other reported cases necessitates acting quickly without delay. Immediate recognition and analysis of the language disturbance and verbal perseverative behaviour may provide relevant information for differentially diagnosing clients presenting with various cortical degenerative diseases at an early stage.

Notes

The authors would like to thank Karin Geisler, Maura Bayer, and Caroline Brew for their critical reading of this paper, and the colleagues who provided the two reviews of this manuscript for their constructive comments and suggestions.

1. Perseveration is defined as the unintentional production of a previously spoken, heard, written, or read linguistic form/language unit or action unit.

2. AP was tested by a psychologist 2 days after the third language testing session. The results from this session were briefly summarised by the psychologist as follows: AP received a score of 7 on the Mini Mental State Exam. She was disoriented for time and location. (For the language interview (first test session) she was able to supply her address and town.) Her short‐term memory was mildly to moderately impaired. However, her long term memory (recall) and retrieval of information was more severely affected. Her visual‐motor coordination was not impaired. She could name a few common everyday objects. On a functional psychosis scale she showed a mild clouding of consciousness and she revealed marked frontal signs.

3. In the hospital where AP was a patient, speech therapists from the Speech Clinic also performed the AAT with AP two days after we tested her. Very similar scores were obtained on all the subtests.

4. Moen's (1993) definitions of echolalia and palilalia are cited here for the reader unfamiliar with the distinctions: “Echolalia: “the automatic and compulsive repetition of words by the patient in the absence of the understanding of their meaning” Brain, 1965, p. 105); “a reflex automatism of verbal response, providing an empty, stereotyped quality to speech” (Hecaen & Albert, 1978, pp. 74/75); “repetition of the investigator”s utterances with or without mild changes in position and choice of words” (Huber, Poeck, & Weniger, 1982, p. 80).”Palilalia: “the involuntary repetition two or more times of a terminal word, phrase or sentence” (Critchley, 1970, p. 201); “a disorder of speech characterized by compulsive repetition of a phrase or word that the patient reiterates with increasing rapidity and with descrescendo of voice volume” (Boller, Boller, Denes, Timberlake, Zieper, & Albert, 1973, p. 1117).” These definitions are quoted from Moen (1993, p. 2). Critchley (1927–1928) describes the term palilalia as “… a variety of reiteration, whereby the patient repeats many times a word, phrase or sentence which he has just spoken. In addition the speech tends to be uttered more and more quickly and with less distinctness” (p. 23). Seven cases presenting with palilalia are discussed in his report.

5. Wallesch (1990) provides a comprehensive discussion of verbal repetitive behaviour including the phenomena: echolalia, palilalia, and perseveration.

6. The symbol ↗ denotes that AP ends the phrase with a rising intonation.

7. Those parts of the target that are perseverated are written in italics and in the case of single phonemes and parts of words, they are also in bold print. In the text examples the symbol “∼” is used for an unrecognisable sound and the symbol “⇒“ is used for “is produced as”. The arrow is also used to denote the beginning of an additional response by AP to a single item. A correct response is marked by “+”. The notation “‐” is used for a brief interruption and a continuation, and “//” denotes a break‐off with a new start (“//”). Squared brackets “[ .. ]” are used for reconstruction of possible omitted words or comments to the transcript or text.

8. Her name is erased in Figure 5a for reasons of data protection.

9. In German, the days of the week beginning with Sunday are: Sonntag, Montag, Dienstag, Mittwoch, Donnerstag, Freitag, Samstag.

10. In German, the months of the year are: Januar or Jänner, Februar or Feber, März, April, Mai, Juni, Juli, August, September, Oktober, November, Dezember.

11. We consider the “intrusions” as defined by Shindler et al. (1984) to be examples of perseveration. In our data from AP, we have only one explicit example of an intrusion. “For no apparent reason” she suddenly spoke of children in a very negative manner (Ger.: “Gefraster”) in a sentence that was not perseverated.