Volume 83, Issue 3 , Pages 423-426, March 2002
Frontal impairment and confabulation after herpes simplex encephalitis: A case report☆☆☆
Article Outline
- Abstract
- Case description
- Discussion
- Conclusion
- Appendix 1: Details about cognitive rehabilitation techniques
- References
- Copyright
Abstract
Del Grosso Destreri N, Farina E, Calabrese E, Pinardi G, Imbornone E, Mariani C. Frontal impairment and confabulation after herpes simplex encephalitis: a case report. Arch Phys Med Rehabil 2002;83:423-6. We describe the rehabilitation training of a 53-year-old woman with severe confabulatory and dysexecutive syndrome, as well as memory impairment, after herpes simplex encephalitis (HSE). Secondary narcolepsy was also present. Neuropsychologic deficits were detailed through an extensive examination, and specific techniques were used to improve performances in each defective cognitive domain. Improvement of vigilance and attention was reached through appropriate and timed periods of rest, along with attentional tasks of growing difficulty. Different external aids were used to reduce temporal disorientation, to contrast confabulation and inertia, and to overcome memory deficits in everyday life. Their independent use by the patient was implemented through cues that were progressively reduced. Treatment also focused on planning, categorization, and topographic orientation. The patient's family gave constant support during rehabilitation and provided informal training after discharge. The patient was able to regain independence in everyday life at home. © 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation
Keywords: Case report, Encephalitis, Herpes simplex, Rehabilitation
Introduction of early antiviral therapy in herpes simplex encephalitis (HSE) has significantly changed life prognosis in this disease, but survivors are generally affected by severe cognitive deficits, particularly loss of memory and frontal dysfunction with behavioral problems.
Previous reports have indicated that rehabilitation can be associated with achievement of partial independence in activities of daily living (ADLs), with patients being able to return home and sometimes to work.1, 2, 3 More extensive data about rehabilitation techniques in this category of patients are lacking; however, HSE may determine peculiar combinations of cognitive deficits, which need specific intervention.
We present a case of successful rehabilitation training in a woman who, after HSE, showed a severe frontal dysfunction with inertia and pervasive confabulation. She also showed amnesia syndrome and vigilance and attention problems, with secondary narcolepsy.
Case description
The subject was a 53-year-old white woman with 13 years of education and who worked in her own flower shop. She was married and had 2 children. She was in excellent health until January 12, 1997, when she began to suffer from vertigo, nausea, diplopia, confusion, and Jacksonian seizures. On admission to a general hospital, a clinical diagnosis of HSE was made and confirmed by polymerase chain reaction for herpes simplex I DNA in the cerebrospinal fluid. Magnetic resonance imaging of the brain showed areas of increased T2 signal in right insula and temporomesial lobe and bilateral frontobasal cerebral cortex, which were enhanced by gadolinium. Other alterations were in the hypothalamus, thalamus, and pons. T1-weighted images showed areas of low signal intensity, with surrounding edema occupying the bifrontal and right temporal lobes.
Neurologic condition improved in the next months after antiviral treatment, and no seizures were reported after the acute phase.
In late April 1997, she was transferred to our Neurorehabilitation Unit. On neurologic examination, the patient was cooperative but passive, confabulant, and persevering. Diffuse hyperreflexia and extensor plantar reflexes with slight left hemiparesis were evident. She presented with frequent brief involuntary naps while standing, eating, reading, or carrying on a conversation. These episodes lasted about 5 minutes, unless the patient was reclining, in which case she continued to sleep for an hour or longer. She underwent a detailed sleep interview and nocturnal polysomnography followed by a multiple sleep latency test. A diagnosis of secondary narcolepsy syndrome was made.
An extensive neuropsychologic examination of the patient was performed before the starting of cognitive training (beginning of May 1997). The assessment was performed in several sessions, owing to fatigue, impaired sustained attention, and narcolepsy attacks. Table 1 shows test scores in detail.
Table 1: Profile of scores in neuropsychologic evaluations
| May 1997 | July 1997 | Oct 1997 | Mar 1998 | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Cutoff | RS | AS | ES | RS | AS | ES | RS | AS | ES | RS | AS | ES | |
| Mini-Mental State Examination | 24 | 29 | 28 | 29 | 28 | 30 | 30 | ||||||
| Corsi tapping test | 3.75 | 5 | 4.75 | 4 | 5 | 4.75 | 4 | ||||||
| Digit | |||||||||||||
 Span forward | 3.75 | 6 | 5.75 | 4 | 6 | 5.75 | 4 | 6 | 5.75 | 4 | 6 | 5.75 | 4 |
| Span backward | 5 | — | 6 | — | 5 | — | 5 | — | |||||
| Serial position curve | |||||||||||||
| Primacy effect | 5 | 6 | 0.5 | 0 | 16 | 10.5 | 2 | ||||||
| Recency effect | 8 | 27 | 21.5 | 4 | 21 | 17.5 | 3 | ||||||
| Logical memory test | 8 | 20.5 | 18.5 | 4 | 19.5 | 17.25 | 4 | ||||||
| Paired associated words test | 6.50 | 16.5 | 15.75 | 4 | |||||||||
| Corsi Supra Span Tapping Test | 5.75 | 7.54 | 2.51 | 0 | 10.25 | 5.25 | 0 | 14.39 | 9.39 | 1 | |||
| Verbal Fluency task | |||||||||||||
| Phonemic cues | 17 | 19 | 15.5 | 0 | 25 | 21.5 | 1 | 29 | 25.5 | 2 | 30 | 26.5 | 2–3 |
| Semantic cues | 25 | 21 | 18.5 | 0 | 31 | 28.5 | 1 | 35 | 32.5 | 2 | 36 | 33.5 | 2 |
| Attentional matrices | 31 | 27 | 21.75 | 0 | 23 | 17.75 | 0 | 37 | 31.75 | 1 | 39 | 33.75 | 1 |
| Raven Colored Matrices (A, AB, B) | 18 | 32 | 31.5 | 4 | 35 | 34.5 | 4 | ||||||
| Battery for verbal judgments | 33 | 40 | 34.25 | 1 | 56 | 50.25 | 4 | ||||||
| Wisconsin Card Sorting Test | |||||||||||||
| Categories | 0 | — | P | 4 | — | N | 5 | — | N | ||||
| Correct responses | 19/64 | — | P | 45/64 | — | N | 57/64 | — | N | ||||
| Perseverative errors | 19 | — | P | 8 | — | N | 1 | — | N | ||||
| Rey complex figure | |||||||||||||
| Copy | 28 | 36 | 36 | N | |||||||||
| Delayed recall | 6.20 | 11 | 12.98 | N | |||||||||
While performing the cognitive evaluation, the patient showed evident psychomotor slowness, severe apathy, and lack of initiative. Even when understanding the task modality, she remained mute and motionless; repeated stimulation and starting commands were necessary. Apathy and lack of initiative were evident also in everyday life activities. We observed mental rigidity and perseverative behavior.
The presence of severe frontal syndrome was confirmed by pathologic scores in the following tests: Wisconsin Card Sorting Test,4 Verbal Fluency both for semantic and phonemic cues,5 and Attentional Matrices and Battery for Verbal Judgments.6 However, her performance in a test of nonverbal reasoning was normal (Raven Colored Matrices7), and her score on the Mini-Mental State Examination8 was also normal, indicating a relative preservation of general intellectual level.
Short-term memory, both verbal and visuospatial, was unimpaired (digit and Corsi span9). Performance in the digit span backward was normal, indicating adequate functioning of working memory. Nonverbal long-term memory was impaired on a sequential block-tapping learning task (Corsi Supra Span Tapping Test6). The patient's performance on long-term verbal memory tasks was normal (Logical Memory Test and Paired Associated Words10), with the exception of a reduction of primacy effect (serial position curve11).
Partial long-term memory impairment was observed in ecologic settings, with both instances of anterograde and retrograde amnesia. She did not recall daily events, she did not recognize medical doctors or nurses, and she could not correctly explain the occupation of her husband and children. During the assessment, and in everyday life, severe spontaneous and provoked confabulation was evident; for example, she often refused to admit she was in a hospital, claiming she was at home or saying she was there only to visit the examiner who was her friend. The administration of an autobiographic memory questionnaire confirmed her impairment.12 Parareduplicative amnesia emerged during conversation; she claimed that 2 identical inpatient units existed, located in 2 streets with identical names, both in Milan.
Constructive praxis (Rey complex figure copy13) fell in the normal range. Language and general semantic knowledge, such as word meaning, object attributes, category relationships, and school learning, was unimpaired during the evaluation.
The patient showed no emotional reaction to her own cognitive deficits during the psychometric assessment.
The patient began a daily cognitive rehabilitation training (2 sessions every day). Care was taken to ensure that she had an hour nap before therapy to reduce recurrent daytime naps. A rigid weekly routine was discussed with her, then it was written on the cover of a memory book.
The routine reported scheduled cognitive and physical rehabilitation sessions, meals, naps, relatives' visits, and her visits to home. She was obliged by therapists, nurses, and relatives to follow this routine strictly, and she was instructed always to carry the diary with her and to note all of her activities with date and time. The patient often denied disturbances involving the use of a memory aid and needed to be repeatedly cued to write in and consult her memory book. She was also unable to select relevant aspects of her environment (writing in her emotions and feelings at the place of daily activities).
Each rehabilitation session began with temporal and spatial reorientation, with the aid of the memory book. She claimed to remember events, but in fact she gave almost always confabulatory answers. She was therefore instructed to read the diary to reconstruct events in the past day or morning. Time was spent to contrast confabulations, showing with written material (eg, clinical chart, yellow pages, city map) or logical deduction that her statements about date, place, and biographic history were wrong. To contrast her severe apathy, a timer was used: the patient was given a fixed time to complete actions in everyday life, eg, 10 minutes to go to the toilet and to be ready for therapy session. The timer use also prevented the patient from falling asleep. As in neuropsychologic assessment, starting commands were used when necessary.
Inpatient training was aimed at ameliorating the following cognitive domains: selective and sustained attention, temporal and spatial framing, visuospatial abilities, planning, categorization, and associative learning. For details about the rehabilitation techniques, see appendix 1.
The patient's relatives had an active role in the rehabilitation program: we explained to them the neuropsychologic bases of deficits shown in everyday life and informed them about all activities carried on every day, with the aim of allowing the family to contrast the confabulation. Her husband and children were also instructed to talk with the patient about salient family events in recent years to facilitate her relearning of autobiographic history.
The patient could spend some hours at home during the weekend. On those occasions, relatives stimulated her to perform activities useful for improving temporal and spatial orientation and for reducing inertia. For example, they asked her to change over the clothes in the wardrobe to help underline the change of season.
After 1 month of training, the patient showed a significant improvement in everyday life; confabulations were reduced, and parareduplicative amnesia disappeared. She showed no more topographic disorientation in the inpatient unit. Temporal orientation was improved: she was able to use a calendar to know the date and the timer to be ready on time, with minimal cues by therapists and nurses. We also noted a greater mental flexibility, with less perseverations.
The patient was then discharged and continued cognitive rehabilitation at the hospital (2d/wk) following the described program. At home, relatives stimulated her to do housework, despite her apathy, and controlled memory book compilation. Rehabilitation continued until July 10, 1997. During this period, we noted “ecologic” improvement in the following areas: autobiographic memory, temporal and spatial orientation, and sustained and selective attention. She was able to use a timer and a calender independently and to manage personal care independently. Psychomotor slowness and apathy was reduced. The patient began to show emotional reactions to her cognitive deficits. A psychometric assessment, performed at this time, showed improvement in frontal tasks (table 1).
In the following months, the patient's family continued to use the strategies previously planned with therapists and to assist her in home care and community affairs.
In October 1997, a neuropsychologic evaluation was repeated, showing a further improvement in frontal tasks (particularly at the Battery for Verbal Judgments6). Nonverbal memory was still impaired (table 1). A new formal training for the patient was felt unnecessary because of the improvement shown at formal tests and reported by her family in everyday functioning. Counseling and suggestions to the family were reinforced.
At the last follow-up control (8 months after formal training ended), the patient showed good insight about her health and cognitive conditions; she showed a great improvement in neuropsychologic functions, with only a mild residual slowness in performing psychometric tasks and ADLs, despite persistence of narcolepsy episodes (table 1). The improvement was mirrored in her behavior at home, particularly in managing housework and shopping, and in remembering recent autobiographic and familiar facts. Relatives also reported a less rigid and perseverative attitude. She did not go back to her work.
Discussion
The aim of this report is to suggest practical strategies to treat cognitive sequelae caused by HSE.
A first important problem to solve in our case was represented by vigilance impairment. The strategy used to reduce this problem was to allow appropriate and timed periods of sleep and rest before and between the rehabilitation sessions. A vigorous approach to improve temporal and topographic orientation and to contrast confabulation represented the most significant part of the training program at beginning of rehabilitation because we considered that this represented a prerequisite to treat other cognitive disturbances. We stress the importance of planning and following a rigid weekly routine and using external aids to improve orientation, as well as the aid of the family.14, 15 As far as confabulation reduction is concerned, the exploiting of residual reasoning abilities and the intensive use of written material were the mainstay of the patient's therapy.
Another prerequisite for developing cognitive strategies and improving independence is the improvement of sustained and selective attention.16, 17 At the beginning of the training program, the patient was unable to maintain attention for longer than 10 minutes; at the last follow-up examination, attentional capacities were largely improved.
Inertia was another obstacle in performing any activity during the first period. This problem was first approached with repeated verbal stimulation and commands. Afterward, external aids, such as the timer, were introduced, initially with a verbal cueing by therapists; the cueing stimulus was then progressively reduced and eventually removed.2
Frontal functions represented another main target of rehabilitation: a deficit in frontal functions is very pervasive, influencing all aspects of rehabilitation and everyday life. Activities aimed at improving planning and categorization in the patient were similar to training used in patients with traumatic brain injury.18
Family and staff participation in the rehabilitation program was fundamental for the patient's recovery. During the inpatient stay, all persons in contact with her took part with respect to the daily routine and stimulated her to use the memory book. Family continued rehabilitation in the home setting and collaborated with therapists to create and use external aids. This is the only way to supply a severely impaired patient with the amount of training needed to obtain improvement.2, 15
Some aspects of the patient's cognitive functioning probably contributed to the success of the program. Even if long-term memory was affected, learning did not rely only on nondeclarative systems because partial use of the explicit route, especially for verbal material, was still possible. General intellectual level and nonverbal reasoning were relatively spared. Moreover, language was intact, allowing her to easily follow verbal instructions and to establish communication with staff and relatives.
The patient's training began in the subacute phase, when spontaneous recovery can still occur. In longitudinal testing, memory performances were stable, indicating that her improved performances in everyday life were because of the implementation of external aids and improvement of orientation obtained with rehabilitation treatment. In contrast to memory performance, frontal functions improved at testing; we cannot estimate the relative contribution of rehabilitation and spontaneous recovery to this improvement. However, when considering severity and characteristics of the initial deficits, it is highly improbable that such a restoration would have been obtained in the absence of intensive training.
Conclusion
Our patient regained independence in most instances of everyday life, also recovering her role as wife and mother. We conclude that cognitive rehabilitation can produce satisfying results in patients with cognitive sequelae by HSE, despite an initial severe impairment.
Appendix 1: Details about cognitive rehabilitation techniques
| Domain | Technique |
|---|---|
| Attention | (1) Performing number and letter matrices of growing difficulties; when patient showed confidence with the task, interference activities were introduced. |
| (2) Identifying specific stimuli in brief paragraphs read by the therapist (auditory attentional task). | |
| Temporal orientation | (1) Remembering specific family events that occurred in the last 7 years, with immediate feedback about her answers. The same procedure was used for recent public events. |
| (2) Recalling simple stories, first spontaneously, then in an ordered way, and finally, in the backward modality from the most recent event to the oldest one. | |
| (3) Cueing memory book compilation and consultation. | |
| Topographic orientation | (1) Reconstructing specific routes in the city map by using public transportation. |
| (2) Locating well-known monuments in a tourist city map, which is associated with recollecting related semantic information. | |
| (3) Drawing simple linear routes with direction changes on squared paper, which was dictated by the therapist. | |
| (4) Cueing (verbally and repeatedly) about topographic references in the hospital and surroundings that the patient could directly observe. | |
| Spatial memory | (1) Hiding an increasing number of familiar objects in the rehabilitation room and asking the patient to remember their exact location with the aid of cues when necessary. |
| Visuospatial abilities and planning | (1) Giving the patient a list of activities to be performed ideally in the morning, along with a simplified map of a city district. The therapist specified the time necessary to perform each activity and to move from 1 place to another. The patient had to choose the route and the sequence of activities to be performed to respect temporal constraints. |
| (2) Giving the patient a picture and mixed test arrangement. | |
| (3) Giving the patient a block design. | |
| Categorization | (1) Classifying pictures and word lists. |
| Associative learning | (1) Transforming abstract concepts into concrete images to strengthen recall. |
| (2) Finding a third element that could link 2 apparently unrelated words. | |
| (3) Learning paired words or pictures by using semantic relationships. | |
| (4) Creating some phrases to remember a list of words. |
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☆ No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit upon the authors(s) or upon any organization with which the author(s) is/are associated.
☆☆ Reprint requests to Elisabetta Farina, MD, Neurorehabilitation Unit I.R.C.C.S. S. Maria Nascente, Don Carlo Gnocchi Foundation, University of Milan, Via Capecelatro No. 66, 20148 Milan, Italy, e-mail: efarina@dongnocchi.it.
PII: S0003-9993(02)13142-X
doi:10.1053/apmr.2002.29646
© 2002 American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.
Volume 83, Issue 3 , Pages 423-426, March 2002
