Ganser's syndrome subsequent to ophthalmic herpes zoster in an elderly woman

Abstract

A case of a 79-year-old woman, who was admitted to a hospital subsequent to a mechanical fall and ophthalmic herpes zoster infection, is presented. She also presented with features of giving approximate answers, fluctuating consciousness, somatic conversion symptoms and probable hallucinations. A presumptive diagnosis of Ganser's syndrome was made. The patient made nearly a full recovery from the above symptoms in about 3 months. However, she continued to have cognitive impairment for which a further diagnosis of vascular cognitive impairment was offered.

Background

Ganser's syndrome was first described in 1897 by the eminent German neurologist, Sigbert Ganser.¹ He described it in three prisoners with the characteristic features of approximate answers, somatic conversion symptoms, clouding of consciousness and perceptual disturbances. For a long time, it was thought that this syndrome was prevalent only in the male prison population; patients exhibiting the syndrome were considered to be malingering as they were thought to have malicious intentions to escape further incarceration. They presented with odd mental health symptoms, which were blatantly false and unbelievable. Later, the concept of factitious disorder (in contrast to malingering) was accepted as the aetiopathological phenomenon for Ganser's syndrome. The differentiation between factitious disorder and malingering can be difficult and there is controversy regarding this nosological differentiation.²

More recently, Ganser's syndrome is considered to be a dissociative disorder having moved into this category from factitious disorder on transition from the Diagnostic and Statistical Manual of Mental disorders-III (DSM-III) to DSM-IV. DSM-IV includes four categories of dissociative disorders: dissociative amnesia, dissociative identity disorder, dissociative fugue and dissociative personality disorder. Ganser's syndrome is classified under another category, which is dissociative disorder not otherwise specified.³ It is defined as ‘the giving of approximate answers to questions’ (eg, ‘2 plus 2 equals 5’) when not associated with dissociative amnesia or dissociative fugue.³ Ganser's syndrome has also been diagnosed in the adult non-prison population as well as in females.^{4 5} This reflects the greater recognition of dissociative states and acceptance that states as those of Ganser's syndrome may be a manifestation of an individual's coping style under stressful circumstances. There is very little published literature on Ganser's syndrome in the older population.⁶

We suspect that there are a large number of patients with Ganser's syndrome in the clinical geriatric setting, and this remains under-recognised and under-reported. We present a case of a 79-year-old woman who presented with symptoms of Ganser's syndrome including approximate answers, somatic conversion, fluctuating consciousness and probable hallucinations. This was subsequent to a fall and a herpes zoster infection involving the trigeminal nerve. She made a spontaneous and full recovery from Ganser's syndrome.

Case presentation

A 79-year-old woman, Mrs. B, was admitted to a dual-care (geriatric medicine and geriatric psychiatry) ward in a district general hospital in the North of England. She had been transferred from a medical ward with a florid case of herpes zoster (shingles), involving the right side of the forehead and scalp extending from the brow to the vertex, matching the neural distribution of the ophthalmic branch of the right trigeminal nerve. She had extensive urticaria, pruritis and pain in the affected region with consequent bleeding. She had also experienced a mechanical fall from a chair and was complaining of lower back and left hip pain prior to admission. This was investigated by the Orthopaedic Team who ruled out any serious injury.

The patient remained in her room and declined participation in ward activities. She would keep her eyes shut and would not open them, even when asked to do so, but it was evident that she was not sleeping. She appeared disorientated, and a baseline Mini Mental State Examination (MMSE) was attempted.⁷ She consistently scored 0/30 on MMSE, which did not match her perceived abilities. Most of her responses were approximate, some of them were as follows:

Question 1: What year is it: 1909

Question 2: And the season: 900

Question 3: And the date: 1909

Question 4: And the day of the week: 990

Question 5: Where are we at the moment: home

Question 6: What town are we in: 909

She stated that her mother and father were alive and that she stayed with them, though the collateral history confirmed that she lived alone. When asked about her son, she initially declared that he was in school; later said that he was a nurse and worked part time (which was found to be true). However, a minute later, she again affirmed that her son was in school, even though not asked about it.

When asked about her age, she stated that she was 3 weeks old; a sentence earlier she had said that she had been in hospital for 3 weeks. She claimed that she could sew and knit and had been doing this on the ward, however there was no evidence that she had participated in such activities.

When asked how many legs a cat has she replied “legs, they are used for walking”. On repeating the same question she said “4”. She was then asked to do simple calculations, on asked what 10+10 was her reply was “7”. When asked how she had arrived at that conclusion, she said “4”. Then she was asked what 2+2 was, to which she replied “10 divided by 4 is 7”. When some subtractions were attempted she again gave approximate replies, for example, 100−5 was “27” and 45−5 was “19”. On repeating simple addition later, she was able to give the correct answers: 2+2 was “4”.

It was suspected that the patient was experiencing visual and/or auditory hallucinations as she was observed at times talking to herself. This, however, could not be confirmed subjectively as the patient would not affirm or deny having such experiences. On one occasion, when asked if she could see or hear anything in her room she said “numbers”. It was thought that this response was related to the mathematical tasks she was given a few minutes ago during the cognitive assessment.

Throughout her stay on the ward, Mrs. B remained placidly cooperative during physical interventions allowing the nurses to change her clothes or the bed linen without any resistance. The nurses also commented that she would make no attempt to use her limbs. They thought that her upper limbs were like stone as she would just let them flail around her hips as if she had no power. However, it was observed at other times that she could pull herself up in her bed by using her hands on the bedrail. She required hoists for all of her transfers.

Mrs. B was observed to have reduced appetite and would not eat most of the food presented to her. She would not make any attempts to engage in a conversation with the nursing staff and resisted all attempts to work with the physiotherapist who tried to help her walk.

Mrs. B's son informed that there had been a sudden and quick deterioration in her motor abilities about 6 months prior to admission. Prior to that she was walking independently. For the last 6 months, she needed help to move around the house and would use a wheelchair to mobilise, however, on some occasions, she would get out of the chair and walk to the toilet on her own. He mentioned that premorbidly she was an active woman who participated in various community activities. She had no significant medical problems prior to this admission. He did not think there had been any depressive symptoms prior to admission. There was no suggestion of anhedonia, lack of energy, poor concentration, low mood, sleep disturbance or any suicidal ideation. He felt that she was experiencing some difficulty with her activities of daily living (ADL) but was still managing well enough to allow her to live mostly independently. Community care staff did assist her twice daily due to her poor mobility prior to admission.

The patient's son also described her as a woman whose personality had changed since the death of her husband a few years ago. She had started to demand more and more from her friends and family with an expectation that she be cared for. Apparently, her husband died of a serious illness; Mrs B was the main carer at the time.

Mrs B's son also observed his mother giving approximate answers to him during the current admission, for example, when he had asked her to identify his wife she was able to give her first name but gave an absurd surname that was neither her married nor her maiden name. She was not able to give a clear and understandable reason for why she had chosen that surname.

There was no significant psychiatric history. Personal history was unremarkable.

On repeat cognitive testing, about 2 weeks after admission, the patient scored higher on the MMSE, achieving 9/30, losing points on orientation, recall, repetition, language, three-stage command and visual perception. Interestingly, she continued to display approximate answers on the orientation tasks. She believed that it was 1890 even though she was able to judge her age correctly and was able to remember her year of birth.

Investigations

Mrs. B underwent systemic examination including a neurological assessment that was unremarkable except for a positive straight leg raising test on the left side, although the patient complained of pain on the right side.

She also underwent blood tests including full blood count, urea, electrolytes, liver function tests, thyroid function tests, blood sugar, glycosylated haemoglobin, lipid profile and prolactin, which were all found to be within normal limits. A urine microscopy and culture was reported normal. A CT head revealed moderate periventricular ischaemia with an established right frontal infarct. A lumbar puncture was considered to rule out zoster encephalitis. However, it was decided not to perform this invasive procedure for reasons of obvious shingles and the fact that it would have been technically extremely difficult because of her lumbar spondylosis and thoracic kyphosis. In addition, her mental state was disturbed. Therefore, it was considered inappropriate to persuade her under best interest principle to have the procedure.

During assessment in the hospital, depression rating scales were applied; she scored 1 out of possible 46 on the Beck Depression Inventory-II (BDI-II) and 21 on possible 42 on the Hamilton Depression Rating Scale (HDRS).^{8 9} It was noted that on the HDRS she scored very highly on somatic symptoms and had null scores on the cognitive symptoms of depression.

A neuropsychology opinion was sought to rule out a dementing illness. The psychologist, however, could not complete the assessment as the patient behaved in a ‘bizarre manner’ giving approximate answers.

Differential diagnosis

A working diagnosis of Ganser's syndrome was made. In addition, there was a suggestion of possible cognitive impairment as observed by the low MMSE scores, fluctuating cognition and vascular changes on CT scan. Hence, an additional diagnosis of vascular cognitive impairment was offered.

Delirium was considered as a differential diagnosis to explain her fluctuating consciousness level but, as she displayed additional symptoms of approximate answers and conversion symptoms, this diagnosis was considered inadequate to explain all of her presenting features. Depression was also considered as a differential diagnosis given her poor appetite, poor memory and lack of interest. However, she scored very low on the BDI, albeit higher on the HDRS on the somatic subscale. This could be interpreted as due to the somatic features of her Ganser's syndrome rather than a depressive episode.

Treatment

The diagnosis of Ganser's syndrome was discussed with the patient's family and the staff looking after her. The staff were asked to encourage and motivate Mrs. B to participate in various activities and to invite her to perform her own self-care. However, no significant improvement in her presentation was observed in the 8 weeks in hospital. She was then transferred to a residential care home. Medications at the time of discharge were as follows: amitriptyline 10 mg nocte; diltiazem 180 mg mane; aspirin 75 mg daily; atorvastatin 10 mg nocte; perindopril 2 mg mane; and senna 7.5 mg mane.

The amitryptyline was started for her postherpetic neuralgia with some benefit. She had also been offered gabapentin for the neuropathic pain but it made her more sedated and therefore was stopped after a few days of initiation.

All of the cardiovascular medications had been started prior to this admission by her general practitioner.

Outcome and follow-up

A visit to the care home 6 weeks after discharge revealed that the patient had made a remarkable recovery in terms of her motor abilities. She was working closely with the physiotherapist and making attempts to walk with a walker. She was lucid most of the time and there was no difficulty in conversing with her. She also participated in physical and mental activities organised by the care home. She still needed some assistance in most ADLs, however she was eating well and had been gaining weight. There were some reports of incontinence and she needed some assistance with her personal hygiene. However, there was no longer any need for hoists, which she had needed in hospital.

On mental state examination, she was appropriate and lucid. She recognised the author but could not recall his name or where she might have seen him. She was happy with the current accommodation and seemed pleased with her ability to participate in the various activities. She no longer experienced pains on her forehead but itchiness in her right eye continued. She denied any perceptual disturbances, and there was no objective evidence of any abnormal perceptions. Cognitively, she had improved further, her MMSE scores were 13/30. Her registration was 3/3, attention and concentration were 2/5 and recall was 0/3, which did not improve with cues. She demonstrated visuospatial as well as frontal executive dysfunction on the clock face and the pentagon test. Her recall of past events leading up to the hospital admission was relatively good but memory subsequent to admission was poor.

Discussion

Case reports of Ganser-like states in the adult population have been published^{4 5} but it is rarely reported in the elderly population.⁶ Our elderly patient presented with late onset Ganser's syndrome exhibiting approximate answers, somatic conversion symptoms and probable hallucinations subsequent to a herpes zoster infection. It could be argued that the approximate answers were a form of perseverative deficits as commonly seen in dementia syndromes. However, most of her answers were “approximate” rather than perseveration as it was felt that she knew the answer but just “passed by” (vorbeigehen), a phenomena aptly described by Goldin¹⁰ in his review of Ganser's syndrome done more than 50 years ago.

It has been reported that patients displaying features of Ganser's syndrome make a good recovery.^{4–6 11 12} Our patient also recovered from the approximate answers and somatic conversion symptoms subsequent to the healing of the herpes zoster infection. However, she continued to display significant cognitive impairment, which could be explained by probable vascular dementia.

A review of literature showed that most patients with Ganser's syndrome have some but varied organic aetiologies, for example, head injury, dementia (specifically frontotemporal dementia and Huntington's), alcoholism, epilepsy, stroke and cerebral infection.¹³ A positron emission tomography study performed on a young patient exhibiting the syndrome after suffering severe, asthma-induced hypoxia revealed hypometabolism in the bilateral occipital and posterior temporal and parietal lobes.¹⁴ In our patient, there appeared a temporal association between the onset of approximate answers and herpes zoster infection. However, her somatic conversion symptoms predated this symptom, which perhaps reflected some ongoing psychological distress. The periventricular ischaemia might also be considered a predisposing factor in this case. There were perhaps some features of underlying cognitive impairment prior to the admission but this was not picked up in the community maybe because of a lack of contact with services.

The diagnosis of Ganser's syndrome in this patient enabled the staff and the family of the patient to understand her presentation and provide support without any discrimination or prejudice. It needs to be noted that somatic conversion symptoms and approximate answers may sometimes be misinterpreted as malingering, which can lead to difficult dynamics and suboptimal care.

Learning points.

• ▶Ganser's syndrome should be considered as a diagnosis where a patient presents with somatic conversion symptoms and approximate answers.
• ▶The syndrome can be found in the elderly.
• ▶Ganser's syndrome may be associated with physical or psychological stress.
• ▶Most symptoms resolve with resolution of the precipitating factors.