Meningioma and psychosis – cause or coincidence?

Meningiomas are the most commonly encountered predominantly non-malignant brain tumours in adults. They are often clinically asymptomatic for years and require no intervention. Certain types of meningiomas are frequently associated with psychiatric symptoms. However, initial surgical and psychiatric intervention may not result in complete psychiatric symptom remission requiring longer term management. Here, we describe a case of meningioma in a patient presenting with concurrent severe psychotic symptoms associated with risk. Relevant recent literature is presented along with management strategies.

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Brain tumours of all types may be associated with a diversity of psychiatric symptoms, at times complicating the clinical picture and obscuring clarity of diagnosis. Although psychiatric symptoms are a common feature of primary and metastatic brain tumour symptomatology, reported in 50–78% of all tumours, occasionally, psychiatric manifestations may be the only presenting feature of a tumour.1,2 Some studies have found an association between tumour location and psychiatric features such as anorexia and hypothalamic tumours, psychoses with pituitary tumours and cognitive deficits with thalamic tumours. However, other tumour locations and types can present with variable psychiatric symptoms, the most commonly
occurring of which are mood disorders (36%), memory symptoms (24%) and psychosis (22%).3     

Meningiomas are thought to derive from the meningothelial cells of the arachnoid layer and are usually non-malignant and slow growing. They account for 37% of all brain tumours in adults.4 Therefore, among brain tumours in general, meningiomas are encountered relatively more frequently in psychiatry (especially old age psychiatry) and neurology settings. Autopsy and imaging studies have estimated subclinical meningioma rates of up to 2.8% in women.5,6 Further data demonstrate a more than twofold higher incidence among females. Atypical and malignant meningiomas comprise a small fraction of the total (5%) and have a slight male predominance.7     

Olfactory groove meningiomas comprise around 10% of all meningiomas. The pathogenesis of this type of meningioma has been linked to the sex hormone receptors oestrogen and progesterone, which are found in 30% and 70% of tumours respectively. They are found predominantly in women with a peak incidence between the ages of 40 and 60 years.8 Olfactory groove meningiomas can present with both physical and psychiatric symptoms. Anosmia is often an early and under-reported symptom. Headache and visual symptoms are presenting symptoms in up to half of patients. Less commonly, urinary incontinence and gait ataxia can present later in the disease course and are less likely to resolve on removal of the tumour.9 Mental state symptoms occur in around 60–70% of patients with olfactory groove meningiomas.10 Patients are often referred directly to psychiatry services for treatment of an assumed primary psychiatric disorder. This can delay the time to surgical opinion. In terms of frontal lobe symptoms, depression, apathy and psychomotor retardation are more common than personality change and disinhibition, although the latter are also observed.2

Case presentation

A 72-year-old woman attended the GP with her son, who gave a four-week history of his mother telling the family that she was going to marry a well-known television personality with whom she had no previous contact. She had worked until the age of 71 years; her primary occupation was as a hairdresser. She lived alone and had retired to a relatively isolated village with little social contact. She had put her house up for sale with the intention of moving to the county where the TV personality lived, several hundred miles from her home. Her son also expressed concerns that the woman was ‘talking to people who were not present’. The General Practitioner assessment of Cognition (GPCOG) screening test was carried out. The patient scored 7/9, indicating equivocal cognitive impairment. Blood tests at the time of referral indicated mildly raised calcium and platelets but were otherwise normal.     

An initial assessment was completed by a community psychiatric nurse, including the Addenbrooke’s Cognitive Examination-III (ACE-III). The ACE-III score was 77/100 with points lost in the following domains: memory (15/26), language (24/26), visuospatial (14/16), fluency (6/14). A CT head scan was requested, and a psychiatrist appointment made. However, shortly before the psychiatrist assessment, the patient was admitted to hospital having fallen down the stairs at home sustaining fractures to her cervical and thoracic spine. Assessment by the liaison psychiatry team indicated that she had fallen at night while attempting to move around her house in the dark in an effort to not wake the TV personality, whom she believed was living with her. While in hospital she had experienced intermittent visual hallucinations of ‘dust’ falling through the air, although this had resolved on discharge.     

Neuroimaging was recommended and undertaken as follows:

– A CT head scan completed as part of the emergency medical assessment indicated a large frontal mass, more pronounced on the left, with extensive perilesional oedema. There was complete compression of the anterior horn of the left lateral ventricle and significant compression of the anterior horn of the right lateral ventricle. There was minimal midline shift to the right.

– A subsequent MRI brain indicated a mass of 5cm x 4.5cm x 4.7cm arising from the olfactory groove, thought to be an anterior cranial fossa meningioma. The lesion exerted significant mass effect with cortical buckling, lateral displacement of the medial aspects of the frontal lobes, posterior displacement of the genu of the corpus callosum and displacement of the lateral ventricles and anterior cerebral arteries. There was also surrounding vasogenic oedema. No atrophy was noted.

The patient was then referred for a neurosurgical removal pre-operative assessment and was discharged home in advance of the planned surgery.     

In the interim, psychiatric assessment took place and indicated an approximately nine-month history of a delusional belief of a romantic relationship with a male TV personality, originating shortly after the patient had received one of his books as a gift. This was completely out of character. She described the TV personality as having knocked at her door one evening, following which they had entered into a relationship, which had become sexual in nature. She spoke warmly of the companionship the relationship gave her and resisted any efforts to suggest it may be delusional in nature. She also spoke of the support the TV personality had offered since the diagnosis of the meningioma, that he was familiar with the neurosurgeon and had requested special treatment for her. She reported experiencing occasional visual hallucinations of family members in her home, becoming irritated when they would not speak to her. There were also two episodes of her being verbally aggressive to strangers in her neighbourhood without provocation.     

There was no evidence of depression, rather she was notably humorous and jovial in all clinical encounters with some grandiosity. She was tangential in conversation at times and difficult to keep on topic with no evidence of dysphasia. The patient’s family reported no concerns about her cognition. Aside from the delusional beliefs, there were no other changes in her personality, judgement or behaviour. Prior to assessment, she was independent in all activities of daily living, including driving.     

There was no past psychiatric history. Her past medical history included Crohn’s disease and hypertension. There was no history of epilepsy, respiratory or cardiac problems. Her mother had been diagnosed with possible dementia (of uncertain type) at the age of 62 years. There was no family history of delusional or psychotic disorders. Prescribed medications included bendroflumethiazide, lercanidipine, atorvastatin, fluticasone nasal spray, and as required co-codamol. 

Case management

Given that there was a possibility that the delusional beliefs were secondary to the meningioma, it was agreed with the patient and her family to further assess her mental state and consider the need for antipsychotic treatment only after surgical removal.     

The tumour was removed successfully without complication. A WHO Grade 2 meningioma in the olfactory groove was diagnosed and confirmed with histology reporting. She was advised by her neurosurgical team not to drive, due to concerns about her judgement of the associated risk.     

Nine days after the successful surgery, the patient completed neuropsychological testing as an inpatient. She was noted to be distractible, with relative preservation of performance on tests of auditory attention and language. Scores were below expected levels on tests of immediate and delayed memory (less than 1st centile and 12th centile, respectively), processing speed (5th centile) and executive functioning (at or below 1st centile). All other assessments were within the expected range. These scores did not correlate with her daily cognitive functioning and there was no evidence of decline in activities of daily living that would be consistent with a dementia. It is possible that her cognitive testing performance may have been adversely affected by her recent operation and recovery at the time of testing. Therefore, ongoing monitoring of cognition was decided. Her mood was noted to be better than would be expected, given the circumstances. She scored 0/40 on the CORE-10 mood scale. The main differential diagnoses were tumour-induced erotomania or primary erotomania. The possibility of frontotemporal dementia was considered but there was
insufficient evidence at the time to diagnose dementia. There was no evidence of another psychiatric or iatrogenic cause.     

In the weeks and months following discharge from hospital, the patient cancelled several planned psychiatry appointments and was challenging to engage in community care planning. Telephone contact, undertaken to ensure a level of monitoring, indicated that she remained notably jovial and she reported having seen less of the TV personality. However, she remained fixed in her erotomanic delusion. She considered moving closer to her family members to reduce her social isolation.     

This was an unusual case of a significant physical health condition presenting together with concerning psychiatric symptoms. It was unclear as to exactly what extent the meningioma was contributing to the psychiatric symptoms. Several other aspects of this case created complexity, such as her isolation, lack of meaningful activities, generally poor engagement with community psychiatric care planning and the risk of potentially unwise decisions as a consequence of her psychotic illness. She was also vulnerable to potential exploitation. A decision was made for further psychiatric review under the ‘best interests’ provisions of the Mental Capacity Act with consideration of antipsychotic and psychological treatment. Community team psychoeducation and engagement work was completed together with efforts to improve the patient’s structured week and reduce isolation. This proved challenging due to her poor engagement because of lack of insight.     

More recently, in the three months following her surgery, she appeared less preoccupied and less distressed with less delusional intensity. The delusional ideation remained, however. There were no serious or imminent concerns regarding vulnerability, unwise decisions, aggression and self-neglect as was previously the case though these risks needed monitoring. Given her persistent psychotic symptoms, risperidone had been offered as a treatment option versus waiting for potential further beneficial effects from surgical treatment. The patient decided on a ‘watch and wait’ approach to observe for further improvements post-surgery, which her family supported. Efforts have been made to engage her in further formal cognitive assessment without success. This will continue. Her family supported ongoing encouragement of community engagement.     

Since her surgery, there have been insufficient grounds to admit the patient under the Mental Health Act for compulsory psychiatric treatment. However, this remains a possibility should her mental state deteriorate and risks increase. An initial antipsychotic (risperidone) has been identified should this be required. In the meantime, she remains sufficiently accepting of monitoring via the mental health team home visits though she intermittently declines this. Her limited family members provide a level of support. If her psychotic symptoms become severe and she refuses community treatment with relevant risk thresholds and criteria reached, the clinical team plan is to assess her under the Mental Health Act.

Management summary

Meningiomas

Small meningioma tumours discovered incidentally and which are without associated symptoms can be managed with regular imaging and clinical review. Prognosis is usually positive for surgically treated meningiomas (90% for Grade 1 WHO tumours). However, over the age of 69 years, there is a reduction in 5-year survival rates (under 85% for Grade 1 WHO tumours – less than 83% for men).4     

Olfactory groove meningiomas can often grow very large before becoming symptomatic. This frequently delays diagnosis and because of this, at a later stage, surgery is often the only management option. Resection results tend to be positive with high rates of complete resection, a low complication rate and low rates of recurrence. Research into post-resection outcomes indicates a high likelihood of improvement in visual loss and mental state changes (even if longstanding).10 The timescale of recovery may not be immediate, however, and may progress slowly. This is due to the presence of resolving oedema, the volume of which has been linked with the presence of psychiatric comorbidity.11 It is therefore important to consider a prospective psychopharmacological and non-pharmacological treatment plan. Appropriate advance investigational work-up (eg blood tests, ECG, physical health measurements) together with concurrent psychosocial management (eg work around engagement and risk minimisation) will avoid a delay in post-op and longer term management. This will ultimately improve prognosis by reducing the likelihood of a delay to psychiatric treatment.

Erotomania

Data including high quality clinical trial evidence on the management of patients with delusional disorders such as erotomania are limited. These conditions are acknowledged to be difficult to treat.12 Secondary erotomania may result from organic pathology (such as tumour or stroke) or a primary mental disorder more commonly. If erotomania results from an underlying mental disorder, such as bipolar affective disorder, eating disorders or anxiety, treatments for these conditions may be used. Psychosis (most commonly delusional disorder) is estimated to occur in around 1 in 20 post-stroke patients.13 Though psychosis due to meningiomas is usually associated with pituitary and temporal lobe tumours, there have been limited case reports in the recent literature describing psychosis in patients with frontal meningiomas though none described erotomania specifically.2,14,15 Where the secondary aetiology is organic, surgical and/or medical intervention may be required. Overall, the management of both primary and secondary erotomania necessarily involves a pragmatic combination of pharmacological, psychosocial and risk management strategies as part of the psychiatric
care plan.16     

Psychosocial interventions focus on a staged strategy, developing a therapeutic alliance and understanding of the psychological risk factors that have contributed to the origin and maintenance of the erotomanic delusion (such as isolation, stress and low self-esteem). Psychosocial intervention aims to reduce these risk factors. This can be achieved with psychological cognitive techniques such as cognitive behavioural therapy (CBT), which aim to counteract the psychosocial factors that sustain the delusion.17 In addition, strategies based on education and other interventions are employed to minimise risks such as distress, vulnerability and criminal behaviour.     

Psychopharmacological measures focus mainly on the use of antipsychotics, which help attenuate delusions and reduce agitation or associated dangerous behaviours. SSRIs may be used to treat secondary depression.18     

More recently, there has been limited evidence that atypical antipsychotics such as risperidone may be helpful in the treatment of delusional disorders more widely, including erotomania.19 There have also been positive reports of effective treatment with clozapine.16 However, due to potential significant side-effects and monitoring required with clozapine (especially in older adults with comorbidity), this option would need to be carefully considered as a last resort, for example, following a lack of success with other antipsychotic options and with sufficient risk evident to justify the treatment. As atypical antipsychotics would be more acceptable to patients than typical antipsychotics, it would seem to be reasonable to treat an erotomanic delusional disorder using those medications that have been found to have good efficacy and tolerability in other psychotic disorders.12 Examples include risperidone, olanzapine, quetiapine and aripiprazole. Prior to modern atypical antipsychotic use, pimozide had traditionally been used, particularly in North America. However, evidence for pimozide is limited and the side-effect profile is less favourable.19     

Hospitalisation may, of course, be required if an individual becomes challenging to treat in the community and a danger to themselves, to the object of their affection or to anyone else. In treatment-resistant cases, ECT may provide remission from delusional beliefs.18

Discussion

This case described an unusual presentation of symptoms of an erotomanic delusional disorder on a background of a large frontal lobe meningioma arising from the olfactory groove. Associated symptoms included brief, temporary visual hallucinations and isolated cognitive testing deficits in the days post-op with no daily living activity decline. Brain tumours are associated with a range of psychiatric symptoms, even in the absence of localising neurological signs. Meningiomas of the olfactory groove have been associated with psychiatric disturbance, including memory loss and personality change.20 Classically, frontal meningiomas have frequently been associated with symptoms such as depression, anxiety, treatment-resistant depression with delusions, apathy, irritability, disinhibition, impulsivity and mania.2 The presentation in this case has illustrated some expected psychopathology but lacked others.     

The treatment of this patient presented several challenges. It was difficult to differentiate diagnostically between secondary psychiatric symptoms arising from the meningioma per se, the effects of the meningioma and those psychiatric symptoms that may have arisen coincidentally. The patient in this case had several risk factors for a primary erotomanic delusional disorder, including age, sex, marital status and social isolation.18 De Clerambault’s original definition of primary erotomania specified an absence of hallucinations.21 Our patient had reported experiencing hallucinations, albeit briefly. Although there are limitations to the original diagnostic criteria, the psychiatric symptoms of this patient extended beyond even the ICD-10 criteria for a delusional disorder and this impacted on management considerations.22,23      

Intuitively, the surgical removal of the meningioma may have been expected to result in a complete resolution of psychiatric symptomatology if the tumour was the primary cause. This does occur in some cases. One study indicated complete or partial resolution of psychiatric symptoms in 85% of cases of supratentorial meningioma.24 However, counterintuitively, psychiatric symptoms can persist or even worsen some time after surgical intervention,2 necessitating back-up psychopharmacological, pharmacological or psychotherapeutic approaches. Prognosis in these cases tends to be similar to that of tumour-free patients. In this case, it was decided to monitor cognitive functioning over time.     

The nature of the associated symptoms can also affect management. In our case, the patient’s poor insight into her delusional symptoms inhibited more timely treatment commencement and desired assessment and follow-up. Her presentation also required the use of appropriate legal frameworks to ensure appropriate treatment and management to enable recovery.

Conclusion

Though significant organic brain lesions, such as tumours, often present with neurological symptoms, they can present initially solely with psychiatric symptoms. In addition, psychiatric complications frequently present during or later in the disease course and require prompt management. Meningiomas, in particular, are distinctive in that they can more frequently present solely with psychiatric symptoms. Less commonly, the psychiatric manifestations can be unusual, as in the case described here. Clinicians should consider early and prompt investigations for an organic cause of psychiatric symptoms where there is an atypical or very rapid onset of presentation or where symptoms are refractory to treatment. This will minimise delay in the treatment of both physical and psychiatric manifestations of the tumour.     

It is also important to be aware that psychiatric symptoms related to meningiomas or other tumours may not fully or even partially resolve after tumour excision as might be expected. This was the case with our patient. The psychiatric symptoms may continue due to ongoing cerebral effects of the tumour, eg slowly resolving oedema or chronic cerebral damage caused by the tumour. Therefore, it is important to consider a prospective psychopharmacological and non-pharmacological treatment plan to reduce the likelihood of a delay to psychiatric treatment. In addition, where there is non-engagement due to psychiatric symptoms with associated risks and a lack of mental capacity to decide on psychiatric care and treatment, management under the Mental Capacity Act or, if required, the Mental Health Act, should be considered. However, criteria for detention under relevant legislation may not be met, necessitating effective care planning, risk management and  monitoring while endeavouring to ensure that disengagement is not precipitated.     

The limited evidence for pharmacological treatment of erotomania underlines the need for further good quality data for the treatment of this disorder, which negatively impacts on mental health and quality of life and is associated with an increased risk profile.

Conflicts of interest

None declared.

Dr Byrne is a Consultant Old Age Psychiatrist and Dr Henry is a Psychiatry ST5 Doctor in Old Age Psychiatry, both at Cumbria, Northumberland, Tyne and Wear NHS Foundation Trust.

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