Abstract
Non-psychotic visual hallucinations and illusions (referred to here as NVHIs), such as visual snow, can be indicators of serious underlying pathologies, including Systemic Autoimmune Rheumatic Diseases. In this Patient Perspective article, I recount some of the impacts of NVHIs, and their attribution/misattribution, on my autoimmune diagnostic journeys. I then draw on these experiences, and the literature, to suggest improvements to policies and practice. It is hoped that these types of changes could contribute to NVHI-related diagnostic opportunities being less often missed.
Keywords
Introduction
Delays in diagnosing and treating Systemic Autoimmune Rheumatic Diseases (SARDs) are common and contribute to long-term damage. 1 Causes of delay include the misattribution of neurological symptoms of undiagnosed SARDs to psychiatric disorders or to presumed patient characteristics, such as attributing lupus fatigue to “teenage laziness.” 1 Among the least researched SARD neurological symptoms are non-psychotic visual hallucinations and illusions. Visual hallucinations are defined in the medical literature as perceptions while awake in the absence of an external stimulus, and illusions as distorted perceptions of external stimuli. Non-psychotic visual hallucinations and illusions (NVHIs) are ones with insight. NVHIs is a term I coined to retrospectively describe some of the symptoms included in my medical records (rather than it being a formal diagnosis I was given). It is also suggested here that NVHIs could prove a useful clinical construct (with proposed categories in Supplemental material, Table A). “Simple”/“unformed” NVHIs (the main focus of this article) include for instance light flashes/photopsia and visual snow, and can be highly intrusive but otherwise relatively benign. They can also, however, be indicators of serious underlying pathologies, 2 some of which (as reported in Supplemental material, Table B) are causally associated with SARDs, including encephalopathies, CNS vasculitis, malignancies, retinopathy, and stroke. For example, the risk of stroke in systemic lupus erythematosus (SLE) is between double and triple that in the general population, 3 and stroke can cause visual snow. 4 In addition, Sloan et al, found that 31% of SARD patients (compared to 9% of healthy controls) reported having experienced non-correctable with glasses “visual changes….e.g. visual snow, after images…” more than three times in their lives. 5 Despite their association with SARDs, however, I have been unable to find case reports of what I have called simple NVHIs making a significant contribution to SARD diagnoses. This may indicate that simple NVHIs represent an important and addressable source of missed diagnostic opportunities/clues.
It is not, however, being argued here that the missed opportunity in my case was a failure to recognize my NVHIs as SARD/SARD-related symptoms, as it remains unclear whether mine are. Rheumatologists seen are divided on the question, and 9 years after the symptoms began I am still waiting to see neuro-ophthalmology for a specialist opinion. The personal experience informed contention is instead that for simple NVHIs to fulfill their potential as diagnostic clues in SARDs and other conditions, it may be necessary for patients to feel comfortable to report them, and for clinicians to recognize them as possible symptoms of non-benign pathologies, to be determined in the context of the patient's other signs and symptoms. Findings from the few SARD studies5,6 with a focus on what I have called simple NVHIs appear consistent with some of my experiences (recounted below in the main text and in Table 1 - “Diagnostic journey time-line”). Of particular note, Arunasalam et al, concluded that “understanding … [simple and complex] hallucinations as potential direct effects of SLE could improve attribution, treatment, … while reducing stigma…”. 6 In addition, the SARD diagnostic journey literature identifies a number of general problems (such as with referrals)1,5,7 which applied in my case in relation to simple NVHIs.
Personal Perspective
Having been in good physical health all my adult life, in 2015 I began accumulating rheumatological-type symptoms (Table 1, rows 2–3). In 2016, NVHIs were added, including (to-date permanent) visual snow, after-images, light flashes, spider's web-like squiggly lines and a large gray “blob,” swirling “clouds” and “self-lighting” of the eye when shut, and night blindness (row 4). After a 12 month wait, I saw NHS (National Health Service) ophthalmology who advised seeing neuro-ophthalmology. As I was told this was not available in Wales, I asked for an NHS referral to England, which the local health board refused (rows 9, 11–12). In 2019, I moved health board areas within Wales and in 2022 rheumatology in my local hospital referred me to an English NHS trust with neuro-ophthalmology (rows 13, 17). The trust later said that it never received this 2022 referral, rheumatology said it never received the trust's rejection of its second attempted referral in 2023, and the trust said it never received rheumatology's 2025 third attempted referral (rows 20, 21, 24–27). Thus, 9 years after my NVHIs began, I am still “waiting” to see neuro-ophthalmology. In the meantime, I was diagnosed with anti-phospholipid syndrome (APS) (a systemic autoimmune condition) (row 8), the SARD Sjögren Disease (row 14), and a range of idiopathic diagnoses (eg, cerebral small vessel disease) (row 7). The NVHIs have continued to progress with, for instance, white surfaces now having a pink hue; and I was knocked unconscious in 2022 in an accident arising from the NVHIs (row 18). It remains unclear whether my NVHIs are SARD symptoms or symptoms of an undiagnosed condition, and I have so far received no treatment for them. On the positive side, in 2019 I joined Cambridge University's ongoing SARD studies as a “patient researcher”.
Diagnostic Journey Time-Line. a
Key: C/S = Conditions and symptoms. Hc = healthcare. Coms = Author comments. N-O = neuro-ophthalmology or neuro-ophthalmological. EngNO = English NHS Trust with neuro-ophthalmology. Wales-Rheum = Author's local rheumatology department. Aptts = appointments. NHS = National Health Service. A&E = Accident and Emergency.
It is not possible from the contradictory NHS England and Wales accounts to determine why administratively I have still not seen neuro-ophthalmology, while not having seen them may help explain the uncertainty that surrounds the etiology of my NVHIs. Nonetheless, the NVHIs and how clinicians responded to them do seem to have impacted my autoimmune diagnostic journeys. It may have speeded-up the APS diagnosis as the rheumatologist in 2017 took account of the visual symptoms—along with eg, “obvious ischaemic changes”—in suspecting APS. This suspicion in turn led to autoimmune blood tests which contributed to the Sjögren Disease diagnosis. However, as NVHIs can not be objectively verified (at least without a functional MRI), and clinician knowledge of them is limited, they were vulnerable to initially being dismissed in primary care as imagination/exaggeration or anxiety. This in turn seemed to contribute to the GP also attributing more physical symptoms (such as swollen hands) to anxiety. Despite these uncertainties as to the NVHI's net diagnostic impact, my diagnostic journey, combined with the literature, arguably casts some light on why the diagnostic opportunities NVHIs offer may be missed. In addressing these issues, I focus on five critical elements of the diagnostic process.
History taking and differential diagnoses: More detailed history taking may have reduced the misattribution of the NVHIs. I cannot, for example, recall a clinician asking about the symptoms that arose at the same time as the NVHIs (such as fever and acute cognitive confusion), and which may have pointed towards possible NVHI etiologies. When I did give a history unprompted it generally felt like it went unheeded. For instance, a clinic letter reported “floaters” (a generally benign phenomena) which I had not mentioned but did not mention any of the neuro-ophthalmological symptoms that I had reported. On the other hand, consistent with Arunasalam et al's findings about SLE patient reluctance to report hallucinations, 6 I did not report my hypnogogic [ie, experienced between sleep and wakefulness] hallucinations for fear that doing so would further fuel GP attribution of my symptoms to anxiety. In addition, with the exception of rheumatology, there appeared to be little differential diagnosis. A neurology clinic letter, for instance, recorded “visual disturbance” under “diagnosis” and did not recommend investigations into what might be causing this. Indeed, there appeared to be a general lack of “diagnostic ambition,” with more institutional incentive to see patients and reduce waiting lists than to diagnose them.
Referrals: Problems with history taking and differential diagnosis led to problems with referrals. For instance, the clinic letter which only reported “floaters” (referred to above) appears to have contributed to the GP deciding that a neuro-ophthalmology referral was unnecessary. Recent research found that GPs not recognizing SARD symptoms, refusing to refer, and/or inappropriate referrals (eg, to psychiatry not rheumatology) could be among the principal reasons for delayed SARD diagnoses. 1 As illustrated with my attempts to get a neuro-ophthalmology referral, there are particular problems in Wales. These are on account of a shortage of some specialists, noted in other studies 7 ; and the NHS Wales out of area/“prior approval” policy which entails an apparently chaotic and inequitable process in which clinicians must apply to their local health board for permission to make a cross-border referral, and which the British Medical Association has criticized. 8 In my case, the health board application seemed half-hearted/ironic in that I pushed the GP to make it but he worded it in such a way as might indicate that it should be rejected. In particular, it states: “He has a plethora of symptoms for which he has been investigated very very thoroughly … but still feels he has a connective tissue disease”; and goes on to stress OCD as a factor. Four years later I was diagnosed with Sjögren's, a connective tissue disease.
Coordination and the proactive patient. Arguably, the most consequential problem was that no clinician saw it as their role to drive forward and coordinate the diagnostic process. As “gatekeepers” to secondary care, GPs seem best placed to perform this role but this may not in general occur.1,7 In my case, this meant that the diagnostic journey kept running into the ground. In particular, when MS was ruled out (as the lesions were “more ischaemic than demyelinating”), the GP wrote that there had now been “sufficient investigations.” Compounding these problems was a general lack of coordination between specialisms. As a result, I felt that it was left to me to coordinate and keep restarting my own diagnostic process, such as seeing a rheumatologist privately to find out what might be causing the “ischaemic” lesions. Patients being proactive—eg, suggesting referrals—often leads to the correct diagnosis in SLE. 1 Reliance on patients, however, seems highly inequitable in that those most in need of a rapid SARD diagnosis (such as someone with vascular dementia) may be least able to orchestrate one. In addition, patients in general may not have the knowledge to know what tests/referrals would be most useful nor (as illustrated in this article) the power to ensure that tests/referrals are undertaken/made.
Practical Recommendations
For academics: Increase research on NVHI prevalence. The absence of NVHIs from clinical guidance may contribute to them not being recognized as possible indicators of non-benign conditions. However, the inclusion of NVHIs in SARD guidance requires more data on how prevalent different NVHIs are in different SARDs.
For the Welsh government: Scrap the current NHS Wales out of area/“prior approval” policy. The policy asserts that it ensures equity of access but seems to most disadvantage those on low incomes who cannot afford a private appointment in England.
For Clinicians: Approach diagnosis “as a team endevour,” 9 and learn more about NVHI etiologies. Being told by an ophthalmology nurse that “everyone has visual snow, but some focus on it” could have left me feeling like a fraud if I hadn’t known how rare visual snow is. In addition, believe patient neurological symptom reports. Those reporting multiple symptoms may have a systemic disease, not health anxiety.
One eye open test: Initially, my squiggles etc were attributed to collagen “floaters,” and the blue light flashes to Posterior Vitreous Detachment. It seems possible that these apparent misattributions could have been avoided with one simple question: “Are the patterns/appearances the same with the right eye open and the left shut and the left open and right shut?” That mine are suggests their origin lies with the brain/visual pathway and not the vitreous of the eye.
This article is a I hope honest but inevitably biased personal account, and I make no claims as to the generalizability of the findings. Nonetheless, it may resonate in parts with other people's experiences and perhaps suggest lines of inquiry for future studies, including ones involving representative samples of patients and clinicians.
Conclusions
Simple NVHIs (such as visual snow, illusory palinopsia, and light flashes) if interpreted along with symptoms that arose at the same time can be indicators of serious underlying pathologies. However, my diagnostic journey experiences suggest that limited clinician knowledge of NVHIs and their associations, and issues with history taking and referrals, may contribute to these diagnostic opportunities being missed. In addition, while health service plans 10 tend to focus on care/treatment for diagnosed patients, the SARD literature,1,7 consistent with my experiences, indicates that a timely correct diagnosis is a neglected but equally critical component of quality care.
Supplemental Material
sj-docx-1-jpx-10.1177_23743735261439465 - Supplemental material for Visual Hallucinations and Illusions as Missed Diagnostic Clues in Autoimmune Disease: A Patient Perspective
Supplemental material, sj-docx-1-jpx-10.1177_23743735261439465 for Visual Hallucinations and Illusions as Missed Diagnostic Clues in Autoimmune Disease: A Patient Perspective by Rupert Harwood in Journal of Patient Experience
Footnotes
Acknowledgments
I would like to thank the following people for their help in relation to producing this article: Jennifer Dimond, Eva (Bic Shan) Liu; Dr Alison Porter, and Dr Melanie Sloan.
Any Other Identifying Information
Information redacted for review:
Under “Personal perspective”: “On the positive side, in 2019 I joined [redacted for review] University's ongoing SARD studies as a ‘patient researcher’. ”
This should read: “On the positive side, in 2019 I joined Cambridge University's ongoing SARD studies as a ‘patient researcher’.”
Author Contributions
The shown author is the only author. The four people included under acknowledgements gave invaluable feedback.
Data Availability Statement
Data for this self-study consists of the author's medical records. These are not available to be shared, as it would risk revealing clinician and institution identities, and unnecessarily compromise patient (ie, the author's) privacy. However, the author is happy to try and answer any specific questions relating to the data.
Declaration of Conflicting Interest
The author declares no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Ethical Approval and Consent
The study (which this self-study was a part of) was approved by Swansea University's Research Ethics Committee (approval no. SUMS RESC 2022-0008) on March 1, 2023. Participants gave both written informed consent and then verbal informed consent before being interviewed. The author of this article was the study investigator and so his informed consent was implicit in him writing this article and was not otherwise required.
Funding
The author received no financial support for the research, authorship, and/or publication of this article.
Supplemental Material
Supplemental material for this article is available online.
