A 39-year-old woman with transient convulsions and vision disturbances: a case report

Abstract

Background

Posterior reversible encephalopathy syndrome is a rare neurological syndrome that refers to reversible subcortical vasogenic brain edema disorder in patients with acute neurological symptoms.

Case presentation

Whether there is a direct causal relationship between pancreatitis and posterior reversible encephalopathy syndrome needs further study. We here report a 39-year-old Chinese woman who was diagnosed with pancreatitis followed by vision disturbance. The patient was finally diagnosed with posterior reversible encephalopathy syndrome. On the basis of this rare case, we analyzed the causes of visual disturbance and proposed diagnostic ideas.

Conclusions

For posterior reversible encephalopathy syndrome, early identification and treatment of the primary disease are particularly important. Imaging and clinical characteristics in posterior reversible encephalopathy syndrome are usually reversible.

Introduction

Vision disturbance is a common clinical manifestation of nervous system diseases. In addition to cerebral infarction, intracranial infection, autoimmune encephalitis (AE), migraine, progressive multifocal leukoencephalopathy (PML), porphyria, mitochondrial encephalopathy, lead poisoning, methanol poisoning, carbon monoxide poisoning, etc., can also cause visual impairment. We describe a 39-year-old Chinese woman who was diagnosed with posterior reversible encephalopathy syndrome (PRES) after being hospitalized for pancreatitis. This patient experienced transient seizures and vision disturbance following hospitalization for pancreatitis, and was finally diagnosed with PRES. This case provides a framework for the diagnosis and management of patients with vision disturbances.

Case presentation

Prior to admission (3 hours), a 39-year-old Chinese woman was diagnosed with pancreatitis because of sudden abdominal pain and was admitted to the intensive care unit (ICU) (Fig. 1). After being admitted to the hospital the next day in the early morning, she suddenly had a transient convulsion, which lasted for approximately 10 seconds and relieved spontaneously. After 15 minutes, the patient reported being blind in both eyes. Ophthalmological consultation showed no apparent abnormality.

Fig. 1.

A On day of admission, abdominal computed tomography showing pancreatic swelling; B on day 8 after pancreatitis, abdominal computed tomography indicating pancreatitis; C on day 14 after pancreatitis, abdominal computed tomography indicating pancreatitis and intraperitoneal drainage

The patient had had pancreatitis twice earlier and was discharged after treatment. She suffers from anxiety and takes propranolol or alprazolam when she feels flustered. Moreover, she had been taking venlafaxine for a long time before this hospitalization. During routine physical examination, she was diagnosed with untreated hypertension and hyperlipidemia. She denied a history of other systemic diseases and infectious diseases, as well as family history of psychiatric disease.

When the patient had vision disturbances, her blood pressure was 145/77 mmHg; however, temperature, heart rate, and other physiological examinations (cardiac, pulmonary, abdominal, and skin) were normal. The summary of the patient neurological examination is as follows. She was conscious and able to respond to questions. Her bilateral pupils were equal in size, with a diameter of approximately 3 mm, and direct and indirect light reflexes were sensitive. However, she turned blind as she could not see objects or count fingers even at short distances (< 30 cm), but perceived light. Optokinetic nystagmus was absent, and fundoscopic examination was normal. Her bilateral frontal striae were symmetric without shallowing, and bilateral nasolabial folds were symmetric. Her bilateral pharyngeal reflexes were symmetrically elicited. Her limb muscle strength and muscle tension were normal, and the tendon reflexes of her limbs were symmetrically elicited. Her bilateral superficial and deep sensations were symmetric. Meningeal irritation and pathological signs were negative in this patient.

The admission blood routine showed normal hemoglobin, white blood cells, and platelets. The neutrophil count was 8.68 (normal 2–7.71) × 10⁹/L. Patient’s liver function, procalcitonin, D-dimer, immunologic tests, and thyroid function were normal; hepatitis A, B, and C, as well as syphilis and human immunodeficiency virus (HIV) tests, were negative. The patient’s triglyceride was 78.68 (normal 0.34–2.30) mmol/L, total cholesterol was 10.62 (normal 3.9–6.46) mmol/L, high-density lipoprotein cholesterol was 1.89 (normal 1.29–1.55) mmol/L, low-density lipoprotein cholesterol was 10.18 (normal 0–3.37) mmol/L, potassium (K⁺) was 3.03 mmol/L, magnesium (Mg²⁺) was 0.67 mmol/L, calcium (Ca²⁺) was 1.76 mmol/L, and high-sensitivity C-reactive protein was 277 (normal 0–5.0) mg/L. The admission kidney function and blood amylase were normal, but the day after admission, her blood creatinine was elevated to 105.5(normal 41.0–73.0) umol/L, blood amylase was elevated to 466(normal 0–150) U/L.

Questions for consideration:

1. What is the differential diagnosis for the patient’s transient convulsions and visual disturbances?

2. What diagnostic tests would be most helpful?

The patient’s visual disturbances had sudden onset. The first and most important thing was whether the loss of vision was real. Considering that the patient suffered from anxiety and may have mood swings after being admitted to the ICU, the possibility of hysterical blindness cannot be ruled out. Additionally, the possibility of visual agnosia cannot be ruled out. For patients with hysterical blindness and visual agnosia, the blinking reflex and the optokinetic nystagmus exist. However, in our patient, when the examiner suddenly illuminated the patient’s eye with a strong beam, the patient had no blink reflex.

Since the blindness of the patient was real, we suspected the most reported reason is simultaneous or sequential bilateral posterior cerebral artery embolisms, resulting in bilateral occipital visual cortex area 17 infarcts. Before visual disturbances, the patient had transient convulsions; therefore, epilepsy should be the differential diagnosis. Neuromyelitis optica (NMO) also presents with initial sudden visual disturbances with intraorbital pain. In addition, the fundus examination of the patient with NMO revealed papilledema and serum aquaporin-4 antibody (AQP4) positivity. Intracranial infection and AE can also cause visual impairment. It is worth mentioning that transient convulsion and visual disturbances can also occur in patients with PRES and intracranial venous sinus thrombosis.

Considering that acute ischemic stroke is the most common cause of cortical blindness, head computed tomography (CT) and head and neck CT angiography (CTA) were immediately performed. Intravenous thrombolysis was done with recombinant tissue plasminogen activator (rt-PA) after excluding intracranial hemorrhage. However, the patient’s visual disturbances did not recover after intravenous thrombolysis, and 3 hours after intravenous thrombolysis, the patient underwent a brain magnetic resonance imaging (MRI), which showed no apparent abnormality (Fig. 2 A).

Fig. 2.

A Magnetic resonance imaging showing no apparent abnormality on day 2 after pancreatitis; B Magnetic resonance imaging showing slightly increased signal in the right parietal lobe and in the bilateral corpus callosum (arrows) on day 8 after pancreatitis; C Magnetic resonance imaging showing no apparent abnormality on day 21 after pancreatitis; D Patient’s magnetic resonance venography on day 8 after acute pancreatitis, and digital subtraction angiography on day 21 after pancreatitis, showing that the superior sagittal, bilateral sigmoid, and bilateral transverse sinuses were poorly visualized, the venous returns are fine.

Questions for consideration:

1. How to explain the MRI and CTA findings?

2. What additional diagnostic tests should be performed?

The patient’s head and neck CTA showed no apparent abnormality. Additionally, the patient’s visual disturbances did not improve after intravenous thrombolysis, and the brain MRI, 3 hours after thrombolysis, showed no evident abnormality to rule out acute ischemic stroke caused cortical blindness. However, arteriovenous malformations and venous sinus thrombosis cannot be ruled out.

In addition to epilepsy, NMO, intracranial infection, AE, and PRES, the broad differential diagnosis included PML, Creutzfeldt–Jakob disease, porphyria, mitochondrial encephalopathy, lead poisoning, methanol poisoning, carbon monoxide poisoning, and migraine. Given the differential diagnosis, a lumbar puncture was performed. The patient’s cerebrospinal fluid (CSF) pressure was 300 mmH2O, with normal routine findings and protein as high as 1732.3 mg/L. The patient’s CSF and serum AQP4 antibodies, AE antibodies, and CSF full set of pathogenic microorganisms metagenomic testing were submitted for inspection.

Treatments for pancreatitis had been ongoing, including fasting, fluid therapy, analgesia, anti-infective therapy, reduction of blood lipids, the use of proton pump inhibitors, and somatostatin. Additionally, mannitol was used to reduce intracranial pressure, and 3 days after admission, the electroencephalogram (EEG) showed medium–high amplitude θ waves in the bilateral occipital, parietal, and frontal areas, and 4 days after admission, the patient woke up in the morning and said that she could see objects clearly. She has had no convulsion since her first transient convulsions. Her vision gradually recovered, and in the evening, her eyesight was back to normal levels before the onset of symptoms.

AQP4 antibodies, AE antibodies, and CSF full set of pathogenic microorganisms metagenomic testing results came back 5 days after admission. Only human coronavirus HKU1 was found positive in this patient. Considering that the first brain MRI was too close to the vision disturbances onset, some lesions may not have been shown; therefore, brain MRI and magnetic resonance venography (MRV) were performed 8 days after the admission. Brain MRI showed hyperintense right parietal sulcus and corpus callosum regions on diffusion weighted imaging (DWI) (Fig. 2B). Although the MRV and digital subtraction angiography (DSA) of the patient showed that the superior sagittal, bilateral sigmoid, and bilateral transverse sinuses were poorly visualized, the venous returns were fine (Fig. 2D). We performed and perfected the patient EEG and lumbar puncture again. The EEG result was the similar to 5 days ago and the CSF pressure was 320 mmH2O, with normal routine and biochemical findings.

Questions for consideration:

1. What is the final diagnosis?

2. How did was the diagnosis verified?

The young woman was admitted to the hospital with acute pancreatitis. Her systolic blood pressure was slightly elevated, lipid levels extremely abnormal, and there were sudden transient convulsions and visual impairment. After her blood pressure returned to normal, lipid levels dropped off, and infection index decreased, the vision disturbances recovered. Although the first brain MRI showed no apparent abnormality, the second showed hyperintense right parietal sulcus and corpus callosum regions on DWI. Our final diagnosis is PRES; 20 days later, we performed MRI again and no lesions were observed (Fig. 2C), which confirmed our diagnosis. Figure 3 summarizes the patient’s disease change process.

Fig. 3.

Schematic diagram of the patient’s blood pressure, important test indicators, and condition changes over time. Due to the different units and normal reference range, the schematic diagram is only used to understand the patient’s disease change process

This patient has been followed up for more than a year and a half. Her platelet count has dropped to normal, and she has stopped taking aspirin. She has been taking fenofibrate because of hyperlipidemia and venlafaxine for an anxiety. During follow-up, we measured her blood pressure multiple times, and it was not higher than 130/80 mmHg.

Discussion

PRES refers to reversible subcortical vasogenic brain edema disorder in patients with acute neurological symptoms (for example, seizures, encephalopathy, headache, and visual disturbances) in the setting of renal failure, blood pressure fluctuations, cytotoxic drugs, autoimmune disorders, and preeclampsia or eclampsia [1]. In addition to our patient, there have been sporadic case reports of PRES in patients with pancreatitis [2–4]. PRES is caused by endothelial injury related to abrupt blood pressure changes or direct effects of cytokines on the endothelium, which leads to the blood–brain barrier breakdown and subsequent brain edema [5]. Whether there is a direct causal relationship between pancreatitis and PRES needs further study.

Although brain MRI is useful, scholars have emphasized that PRES is not an imaging diagnosis. It should be diagnosed according to the patient’s clinical symptoms and imaging features [1]. Edema in PRES is usually asymmetric but almost always bilateral. Besides “bilateral,” the most important feature of PRES is “reversibility.” [5]. A case series that include patients with non-reversible damage reported complete recovery in 64–80% of cases in a mean time of 2–8 days. However, it can take several weeks before full recovery is demonstrated [6–9], and on the basis of the results of case retrospective studies, it is impossible to ascertain the sequence of clinical symptoms and imaging in PRES, nor to know the time of clinical and imaging symptoms remission accurately. This case made a detailed record of the patient’s clinical symptoms and imaging to provide a reference for future research.

In a retrospective study, CSF pressure fluctuations in patients with PRES were 180–280 mmH2O [10]. Our patient’s CSF pressure was as high as 320 mmH2O. Although the MRV and DSA of the patient showed that the superior sagittal, bilateral sigmoid, and bilateral transverse sinuses were poorly visualized, the venous returns were fine. Therefore, venous sinus thrombosis was not the cause of increased intracranial pressure. Retrospective studies have shown that the prognosis of PRES is related to elevated CSF protein, and in our patient, CSF protein was as high as 1732.3 mg/L. After the symptoms improved, CSF pressure decreased and CSF protein returned to normal [11].

Conclusion

In this case, intravenous thrombolysis was performed before establishing the diagnosis in the absence of contraindications because it is reasonable in such patients. For PRES, early identification and treatment of the primary disease are particularly important. Imaging and clinical characteristics in PRES are usually reversible; therefore, most patients have good prognosis.

Abbreviations

PRES

Posterior reversible encephalopathy syndrome

AE

Autoimmune encephalitis

PML

Progressive multifocal leukoencephalopathy

ICU

Intensive care unit

NMO

Neuromyelitis optica

AQP4

Papilledema and serum aquaporin-4 antibody

CT

Computed tomography

CTA

Computed tomography angiography

Rt-PA

Recombinant tissue plasminogen activator

MRI

Magnetic resonance imaging

CSF

Cerebrospinal fluid

MRV

Magnetic resonance venography

DSA

Digital subtraction angiography

EEG

Electroencephalogram

Author contributions

Jing Fan: drafted manuscript and made important additions to the data in the manuscript. Taojiang Chen: supervision of clinical care, patient follow-up, interpretation of imaging results, reviewed manuscript for intellectual content, and contribution to the study concept/design. Pian Wang: attending physician, drafting manuscript, and reviewed literature. Tao Hai: reviewed manuscript for intellectual content. Wei Li: reviewed manuscript for intellectual content. Yan Wang: drafted manuscript, study concept/design, and reviewed manuscript for intellectual content, agreeing to be held accountable for all aspects of the work and working to resolve any issues raised.

Funding

Funding source from Sichuan Provincial Health Commission Popularization project 21PJ150; Chengdu high-level clinical key specialty construction project.

Availability of data and materials

The datasets used in the manuscript are available from the corresponding author on reasonable request.

Declarations

Ethical approval and consent to participate

The ethics committee of Chengdu Fifth People’s Hospital approved this study.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

All the authors declare no potential conflicts of interest.