Unusual ego-defence in HIV encephalopathy: a case report
Schalk W. du Toit*, Saxby Pridmore, Mohammad Khan
Professor Saxby Pridmore, Consultant Psychiatrist, University of Tasmania, Department Psychological Medicine, Tasmania, 7010, Australia, Dr. Mohammad Khan, Senior Registrar, Department Psychological Medicine, Royal Hobart Hospital, Tasmania, 7010, Australia.
Emotion focused coping strategies, such as denial and escape-avoidance techniques, might have short-term benefits for patients with chronic illnesses (including those with Human Immunodeficiency Virus), however it is associated with increased psychological distress and depressive symptomology in the long-term. The authors present the case of a 34 year old man, who is HIV-positive and presented with auditory and visual hallucinations, persecutory delusions, somatic passivity, cognitive deficits and executive dysfunction. It is argued that in addition to the demonstrated psychotic symptoms and cognitive deficits, there were unusual responses on mental state examination, which had an ego-defensive function, i.e. the inability to function in his usual manner was perceived by the patient as ego-threatening. This case study illustrates some important aspects of disease progression in HIV/AIDS and its neuropsychiatric complications, as well as interesting psychophenomenology. The authors conclude that although the mental state signs appear unusual and worthy of mention, the underlying psychodynamic processes involved seem to be that of emotion focused coping strategies, which are employed subconsciously as a defence mechanism in order to preserve the patient’s own ego-integrity.
Key words: HIV encephalopathy; AIDS dementia; ego-defence; coping strategy(ies); defence mechanism(s).
In this case report study we discuss the history, clinical findings and diagnosis of a man with HIV (Human Immunodeficiency Virus) encephalopathy, who was admitted to hospital with psychotic symptoms, cognitive deficits and executive dysfunction. We describe aspects of his presentation, mental state examination and relevant special investigations, but also highlight the interesting and unusual psychophenomenology encountered. It would seem that the underlying psychodynamic processes involved in this particular case, is that of emotion focused coping strategies, which are employed subconsciously as a defence mechanism in order to preserve the patient’s own ego-integrity.
HIV encephalopathy is best described as a subacute encephalitis, which results in a progressive subcortical dementia and usually presents without neurological signs. It affects up to 50% of HIV-infected patients to some degree. It is often associated with social withdrawal, low mood, apathetic personality changes, psychomotor slowing and poor concentration. Memory loss is possible, although unusual in early HIV encephalopathy. There can be visuo-spatial abnormalities as well as motor symptoms such as impaired coordination, ataxic gait, hyperreflexia and increased muscle tone (Treisman et al, 2005).
HIV encephalopathy is often a prodromal stage of AIDS (Acquired Immune Deficiency Syndrome) dementia, which tends to be diagnosed when opportunistic infections arise or when the CD4 lymphocyte count falls below 200 cells/ul. By this stage in the illness patients more commonly present with neurological signs (Treisman et al, 2005). Only about 20-30% of patients with HIV will develop AIDS dementia complex.
Mild to moderate cognitive impairment has been reported in about 40% of patients with symptomatic HIV illness, increasing to more than 50% for patients with AIDS-defining conditions (Hinkin et al, 1998). HIV infected patient’s cognitive functions can stabilise or even improve with HAART (Highly Active Antiretroviral Treatment) therapy, but remain more likely to be impaired than the general population (McCutchan et al, 2007).
Illicit drug abuse continues to be a significant risk factor for HIV transmission worldwide. Although it remains much-debated, evidence suggest that HIV-associated CNS (central nervous system) disorders are probably accentuated in drug abusers (Bell et al, 2006).
Mr. A is a 34 year old single, homosexual man. He had a past history of intravenous drug abuse and had been Hepatitis B, C and HIV positive for approximately 15 years. There was no history of chronic liver disease or hepatic failure.
Mr. A’s first admission to the psychiatric department was four years previously. On that occasion he presented with auditory hallucinations and persecutory delusions. These symptoms partially responded, but did not remit with antipsychotic medication, and he was finally discharged to the care of his parents. In the intervening period he had been interstate and claimed to have been working for a few months in the catering business.
He had stopped using illicit drugs a few years earlier, and there was no history of alcohol abuse. There was no family history of schizophrenia.
Mr. A’s parents requested assessment. They reported increasing “paranoia”, “voices”, disorganized behaviour and poor sleep. Mr. A had ceased his antipsychotic medication one year previously, however he remained compliant with his HAART medications. He did not complain of any physical symptoms, and there was no obvious precipitating factor or stressor.
Mental State Examination
On questioning, Mr. A could not explain why re-assessment had been requested. However, he was pleasant and co-operative and reasonably well kempt. His speech appeared normal. There was no subjective complaint and objectively his mood appeared euthymic, however his affect was blunted.
Thought form appeared vague and there were examples of derailment, however he was able to make grammatically correct sentences. Content of thought included the presence of unsystematised persecutory delusions, with Mr. A talking about being “manipulated by others.” He described multiple auditory hallucinations, some in external space. There was evidence of visual hallucinations, with Mr. A saying, “I can see objects leaving my body.“ There was evidence of somatic passivity. Mr. A reported that others were “moving my back” and he had no control over this. There was no evidence of thought block, insertion, withdrawal or broadcasting.
Cognitive testing revealed many deficits. The overall mini-mental state examination (MMSE) score ranged between 20-25/30. Although Mr. A was orientated in place and reasonably orientated in time (frequently missing the date by one), his immediate memory was poor. He could not remember meetings of the previous day and could remember only 2 of 4 new items.
Testing for dyspraxia, showed Mr. A’s inability to correctly copy interlocking tetrahedrons and he performed poorly in copying and remembering the Rey-complex figure. He could not draw a clock face correctly, placing the numbers and hands of the clock in the wrong positions (Figure 1).
Testing for visual scanning, numeric sequencing and visuomotor speed abilities using the Trail Making Test (Part A), showed Mr. A could not follow instructions and instead of linking the numbers, he wrote various numbers on the test sheet.
Executive functioning tests revealed further deficits. Testing for mental flexibility and verbal fluency, Mr. A scored below the 5th percentile on the Controlled Word Association Test (Benton, 1973). When asked to give all the words he knew beginning with the letter F, he said, “food, fuck” and then perseverated on the word “fuck” until asked to stop.
Figure 1: Clock-drawing and interlocking tetrahedrons tests:
Mr. A’s completed clock face (time of 15h10) and his attempt at copying the interlocking tetrahedrons, which is the middle figure. (Note: These figures are not to scale).
Figure 2: The 15-Item Memory Test:
The angular scribble represents Mr. A’s attempt at copying the 15-Item Memory Test on the left. (Note: This figure is not to scale).
When tested for abstract reasoning, and asked to give the meaning of a commonly used proverb such as “The early bird catches the worm”, Mr. A replied “Early in, early out.” (This could be interpreted as another example of perseveration, i. e. continuing to use a word in a context where it is inappropriate).
Other aspects of mental state examination revealed unexpected phenomena. These became more frequent when the tasks became more difficult and included the following:
• When pressed to explain why he was in hospital, and when asked the difference between a child and a dwarf, Mr. A answered with strings of neologisms (a new word created by a patient, often by combining syllables of other words, for idiosyncratic psychological reasons), which were said with a meter suggestive of a foreign language. [We would like to emphasise that neologisms were not part of his normal communication and appeared only with more difficult questions].
• When testing his ability to register new information using the forward 5-digit span test, Mr. A gave the first 3 correctly, but then continued in a confident manner giving a further 4 incorrect numbers.
• Mr. A was asked to remember and then write down the 15-Item Memory Test (Lezak, 1976). It consists of 5 rows of three columns of symbols and is suggested in the assessment of malingering. Mr. A’s attempt was quite bizarre and took the form of an angular scribble (Figure 2). [We acknowledge that in this case no conclusions could be made about malingering, in view of the patient’s immediate memory deficits and evidence of dyspraxia].
• Testing for sequencing ability and when performing the fist-side-palm test (Luria’s 3-stage motor test), Mr. A added a movement by pronating his forearm to tap the lateral (rather than the medial) aspect of his fist on his leg.
• Mr. A’s attempt at completing the Alternating Sequencing Task took the form of a straight line with a curl at the end, which bore no resemblance to the pattern he was asked to replicate.
These phenomena were performed with a serious demeanour and the impression was that Mr. A believed he was co-operating and performing the tasks as requested. Thus, the generation of these signs and the underpinning motivation appeared to have been unconscious.
Systemic and neurological examination were within normal limits, with no focal abnormalities or motor symptoms.
• All relevant blood investigations (including FBC, LFT, U&E, thyroid) were normal.
• CD4 lymphocyte count was within the normal range at 774/µl.
• Lumbar puncture and cerebro-spinal fluid analysis were requested to excluded the possibilities of neoplasms and infective causes. The results were all normal.
• Magnetic Resonance Imaging (MRI) of the brain showed slight prominence of ventricular system, basal cisterns and cortical sulci.
• Magnetic Resonance Spectroscopy (MRS) provides a measure of in vivo brain chemistry and can be a useful tool to discriminate between different types of dementia. However, in this case the results appeared to be equivocal.
o Multivoxel spectroscopy indicated elevated myo-inositol peaks suggestive of dementia.
o Single-voxel spectroscopy showed normal peaks for myo-inositol, creatine and N-acetylaspartate (NAA).
Progress on the ward
Mr. A was eventually discharged from hospital after his persecutory delusions ceased and his auditory hallucinations partially responded to an atypical antipsychotic, Olanzapine 20 mg/day.
The patient presented with insomnia, disorganized behaviour, persecutory delusions, auditory and visual hallucinations. Mental state assessment revealed blunted affect, derailment of thought, neologisms, somatic passivity, visuospatial abnormalities, dysexecutive syndrome (including perseveration and confabulation), immediate memory impairment and poor registration of information.
A most likely diagnosis of HIV encephalopathy with psychosis, was made by excluding other neurological disorders such as opportunistic CNS infections, CNS neoplasms and aseptic meningitis. Hepatic encephalopathy (secondary to Hepapitits B & C) seemed unlikely, in view of previous normal liver function tests and absence of chronic liver disease.
Although aspects of the presentation are typical of that seen in paranoid schizophrenia, it cannot be diagnosed in the presence of an organic brain syndrome. Clearly, Mr. A’s poor compliance with his antipsychotic medication could have contributed to a relapse in his psychotic symptoms. The previous misuse of alcohol and illicit drugs could also be a contributary factor to the cognitive deficits seen.
In describing Mr. A’s psychopathology, various interpretations could be made, each introducing its own lexicon. The neologisms, extra numbers and movements added to the digit span and Luria motor tests respectively, could be considered a form of confabulation. Failure to draw a clock face accurately could represent constructional apraxia or spatial agnosia, and the pronation of the forearm during the fist-side-palm test could represent apraxia.
In spite of the psychotic and cognitive deficits (both of which contributes to the disorganized behaviour), we conceptualized some features e.g. the neologisms, the incorrect but confidently stated digit span numbers and the angular scribbling, as being of a psychodynamic origin. We propose that Mr. A’s inability to function in the usual manner was ego-threatening, and the abnormal behaviour and speech was an ego-defensive process.
According to Freud, ego-defensive mechanisms reside in the unconscious and are usually adaptive. In this case however, Mr. A’s reality testing (an ego-defence refering to the capacity to distinguish internal fantasy from external reality) became maladaptive and unable to deal with changing realities.
There is a tendency for patients to use emotion focused strategies, such as escape-avoidance and distancing, rather than problem focused strategies in encounters where they believe their options for affecting the outcome of their illness are limited (Grummon et al, 1994). Mr. A’s response to some questions during mental state examination, appeared to be reminiscent of denial/escape-avoidance strategies.
When dealing with organic brain disorders there is a tendency to focus on imaging and cognitive test results, whereas the importance of psychosocial and psychodynamic issues and the impact this might have on mental state presentation, is often overlooked.
HIV infection still carries a significant degree of social prejudice and stigma. As the illness progresses patients often start to favour emotional support, rather than informational support (Friedland et al, 1996). They also start to use more emotion focused coping strategies, such as distraction and escape-avoidance techniques. Although this might be beneficial for short-term adaptation to ill health, it has been shown to be detrimental in the long-term (Suls et al, 1985), as well as being associated with increased psychological distress and depressive symptomology (Folkman et al, 1986).
Clinicians caring for patients with HIV/AIDS or any other chronic illness, should be alert to the presence of unconscious defence mechanisms, particularly when interpreting cognitive test results.
Although special investigations and neuroimaging are of course important when cognitive deficits develop, we suggest more emphasis should be placed on providing psychological support, aimed at facilitating problem focused coping skills. Where emotion focused coping strategies already exist, clinicians should aim to ameliorate these by way of promoting positive reappraisal and acceptance of their illness. Patients however, need to have some level of understanding and ability to learn, in order for this rehabilitation strategy to be effective.
We would like to thank Dr. Rainer Goldbeck, Dr. Stephen Bell and Dr. Fiona Summers for their comments and advice on the draft manuscript.
* Corresponding author: Dr. Schalk W. du Toit, Specialty Registrar ST6, General Adult Psychiatry, Royal Cornhill Hospital, Aberdeen, Scotland, AB15 6YP, United Kingdom.
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Copyright Priory Lodge Education Limited 2009
First Published November 2009