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BOHR International Journal of Neurology and Neuroscience
2024, Vol. 2, No. 1, pp. 29–33
DOI: 10.54646/bijnn.2024.17
www.bohrpub.com
CASE STUDY
A case report of posterior reversible encephalopathy
syndrome in a patient diagnosed with emphysematous
necrotizing biliary pancreatitis
Maryam Nazir Ahmad1*, Anandi Damodaran2, Sarah Muhammad Iftikhar3 and Devdutt Nayak Kotekar2
1Specialist Physician, Department of Internal Medicine, Kuwait Hospital Sharjah, Sharjah, United Arab Emirates
2Consultant Neurologist, Department of Internal Medicine, Kuwait Hospital Sharjah, Sharjah, United Arab Emirates
3General Practitioner, Department of Internal Medicine, Kuwait Hospital Sharjah, Sharjah, United Arab Emirates
*Correspondence:
Maryam Nazir Ahmad,
dr_taurean@hotmail.com
Received: 24 November 2023; Accepted: 14 December 2023; Published: 22 January 2024
Posterior reversible encephalopathy syndrome (PRES), a clinical radiological syndrome, is characterized by the
abrupt development of neurological symptoms such as headaches, convulsions, altered sensorium, and visual
problems. PRES has been linked to a number of risk factors or etiologies, including the use of immunosuppressants
or cytotoxins, hypertensive encephalopathy, eclampsia, preeclampsia, and underlying autoimmune diseases.
A 41-year-old female was admitted with acute necrotizing emphysematous pancreatitis complicated by posterior
reversible encephalopathy syndrome. She remained normotensive on presentation and during the hospital stay.
All her investigations were within normal except double-stranded DNA, which came positive. This could explain
the immune-mediated mechanism leading to endothelial dysfunction. This case has been presented for its rarity.
When acute neurological abnormalities occur in the context of systemic inflammatory conditions, such as acute
pancreatitis, it is crucial to take PRES into account.
Keywords: posterior reversible encephalopathy syndrome, emphysematous necrotizing biliary pancreatitis, PRES,
pancreatitis, case report
Introduction
Posterior reversible encephalopathy syndrome (PRES), a
clinical radiological syndrome, is characterized by the abrupt
development of neurological symptoms such as headaches,
convulsions, altered sensorium, and visual problems (1).
Magnetic resonance imaging shows hyperintensity in T2
and hypointense in T1 representing white matter vasogenic
edema. This is mostly a symmetric disorder with asymmetric
variants. Symmetric lesions are typically located in the
posterior cerebral regions. However, PRES is a misnomer
as the edema is also seen in watershed zones other
than parietal-occipital regions, thalamus, and sometimes
in the anterior circulation. Moreover, the syndrome is
not always reversible. Life-threatening complications can
develop such as transforaminal cerebellar herniation and
focal neurologic deficits, especially if prompt treatment is not
initiated (2).
Various risk factors or etiologies have been identified with
PRES such as hypertensive encephalopathy, preeclampsia,
eclampsia, use of cytotoxins or immunosuppressants and also
in underlying autoimmune conditions (3).
Case report
A 41-year-old female was admitted to the surgical ward as
a case of Severe Acute Emphysematous Necrotizing Biliary
Pancreatitis on conservative management. During the course
of the hospital stay, she was intubated for 11 days and
successfully extubated.
29
30 Nazir Ahmad et al.
FIGURE 1 | Initial CT brain showing hypodense changes in the bilateral parieto-occipital regions.
FIGURE 2 | MRI DWI showing diffusion restriction in bilateral parieto-occipital region.
10.54646/bijnn.2024.17 31
FIGURE 3 | MRI flair image showing signal change in bilateral parieto-occipital region.
On the 56th day, it was noticed during surgical
rounds that the patient was not communicating and
she was referred to a neurologist. On examination, the
patient was conscious, awake, making eye contact, not
communicating, not responding to commands. The pupils
were 3 mm reacting to light, and the patient was
spontaneously moving her eyeballs in all directions. She
had no obvious facial asymmetry and was moving all her
limbs to pain. Her reflexes were normal and plantar -
bilateral flexor.
CT brain (Figure 1) and MRI brain (Figures 2, 3)
were done, which showed evidence of posterior reversible
encephalopathy syndrome. After returning from CT brain,
the patient developed 2 episodes of convulsions.
She was treated conservatively with anti-epileptics and
angioedema measures. Routine blood investigations
were normal while autoimmune workup showed
positive anti-dsDNA. Blood pressure remained stable
throughout the admission.
The repeat CT Brain (Figure 4) done after almost
20 days showed a regressive course of PRES. The patient
symptomatically improved and was discharged.
Discussion
The pathophysiology behind PRES is unknown.
Various explanations have been proposed like cerebral
vasoconstriction resulting in brain infarcts, cerebral auto-
regulation failure with vasogenic edema, and endothelial
injury with blood–brain barrier disturbance resulting in
vasogenic edema. This vasogenic theory explains that
development of PRES in cases of accelerated hypertension.
The posterior circulation of the brain is comparably less
innervated with sympathetic nerves, which is most likely the
cause of PRES’s preferential involvement of the posterior
portion of the brain (4, 5).
At least 20% of patients with PRES have normal blood
pressure throughout the course of the disease. Endothelial
dysfunction in these patients is probably caused by immune-
related mechanisms, such as the secretion of inflammatory
cytokines like interleukin 1, tumor necrosis factor-1, and
interferon factor gamma.(5)
To the extent of for knowledge, very few cases of PRES
associated with pancreatitis have been reported. Murphy
et al. and Magno have presented reports of PRES associated
32 Nazir Ahmad et al.
FIGURE 4 | Follow-up CT brain showing regression of previous signal changes.
with pancreatitis due to alcoholism (6, 7). Palmer et al. have
presented a case report of PRES associated with pancreatic
carcinoma (8).
Posterior reversible encephalopathy syndrome should
be taken into consideration when a patient with acute
pancreatitis experiences headache, visual impairment, and
seizures. Acute pancreatitis may cause cytotoxic and
vasogenic edema by damaging secondary cerebral vascular
endothelium through both local and systemic inflammatory
pathway activation (3).
Our patient presented with Severe Acute Emphysematous
Necrotizing Biliary Pancreatitis leading to PRES. She
remained normotensive on presentation and during the
hospital stay. All her investigations were within normal
except double-stranded DNA, which came positive. This
could explain the immune-mediated mechanism leading to
endothelial dysfunction.
Conclusion
This case has been presented for its rarity. When acute
neurological abnormalities occur in the context of systemic
inflammatory conditions, such as acute pancreatitis, it is
crucial to take PRES into account.
To the extent of our knowledge, this is the first case with
emphysematous necrotizing pancreatitis leading to PRES
with complete recovery.
Conflict of interest
The authors declare that research was conducted in the
absence of any commercial or financial relationships that
could be construed as potential conflict of interest.
Ethics statement
As per Emirates health service policy, ethical committee
approval is not needed for case reports.
Author contributions
MA: study design and conceptual development. AD:
study design and conceptual development. SI: data
acquisition and analysis. DK: conceptual development.
10.54646/bijnn.2024.17 33
All authors contributed to the article and approved the
submitted version.
Consent for publication
Written consent for publication is taken from the patient.
References
1. Hobson E, Craven I, Blank S. Posterior reversible
encephalopathy syndrome: a truly treatable neurologic
illness. Perit Dial Int. (2012) 32:590–4. doi: 10.3747/pdi.2012.
00152
2. Zelaya J, Al-Khoury L. Posterior reversible encephalopathy syndrome.
StatPearls. Treasure Island (FL): StatPearls Publishing (2023).
3. Luo J, Zhan Y, Hu Z. Posterior reversible encephalopathy syndrome in a
woman with pancreatitis. Chin Med J. (2019) 132:2265–7. doi: 10.1097/
CM9.0000000000000431
4. Choe S, Ganta N, Alnabwani D, Hechter S, Alsaoudi G, Patel V, et al. A
case report of severe posterior reversible encephalopathy syndrome due
to accelerated hypertension in a young patient. Cureus. (2022) 14:e26918.
doi: 10.7759/cureus.26918
5. Bonavia L, Jackson J, Jurevics R. Posterior reversible encephalopathy
syndrome in acute pancreatitis: a rare stroke mimic. BMJ Case Rep. (2020)
13:e232228. doi: 10.1136/bcr-2019-232228
6. Murphy T, Al-Sharief K, Sethi V, Ranger G. Posterior reversible
encephalopathy syndrome (PRES) after acute pancreatitis. West J Emerg
Med. (2015) 16:1173–4. doi: 10.5811/westjem.2015.8.28347
7. Magno Pereira V, Marote Correia L, Rodrigues T, Serrão Faria G. Acute
pancreatitis and posterior reversible encephalopathy syndrome: a case
report. Acta Med Port. (2016) 29:567–71. doi: 10.20344/amp.7368
8. Palmer K, Weerasuriya S, Whitelaw B, Srirajaskanthan R. Posterior
reversible encephalopathy syndrome in a patient with a metastatic
pancreatic neuroendocrine tumour and ectopic Cushing’s syndrome.
Endocrinol Diabetes Metab Case Rep. (2021) 2021:21–14. doi: 10.1530/
EDM-21-0014

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A case report of posterior reversible encephalopathy syndrome in a patient diagnosed with emphysematous necrotizing biliary pancreatitis

  • 1. BOHR International Journal of Neurology and Neuroscience 2024, Vol. 2, No. 1, pp. 29–33 DOI: 10.54646/bijnn.2024.17 www.bohrpub.com CASE STUDY A case report of posterior reversible encephalopathy syndrome in a patient diagnosed with emphysematous necrotizing biliary pancreatitis Maryam Nazir Ahmad1*, Anandi Damodaran2, Sarah Muhammad Iftikhar3 and Devdutt Nayak Kotekar2 1Specialist Physician, Department of Internal Medicine, Kuwait Hospital Sharjah, Sharjah, United Arab Emirates 2Consultant Neurologist, Department of Internal Medicine, Kuwait Hospital Sharjah, Sharjah, United Arab Emirates 3General Practitioner, Department of Internal Medicine, Kuwait Hospital Sharjah, Sharjah, United Arab Emirates *Correspondence: Maryam Nazir Ahmad, dr_taurean@hotmail.com Received: 24 November 2023; Accepted: 14 December 2023; Published: 22 January 2024 Posterior reversible encephalopathy syndrome (PRES), a clinical radiological syndrome, is characterized by the abrupt development of neurological symptoms such as headaches, convulsions, altered sensorium, and visual problems. PRES has been linked to a number of risk factors or etiologies, including the use of immunosuppressants or cytotoxins, hypertensive encephalopathy, eclampsia, preeclampsia, and underlying autoimmune diseases. A 41-year-old female was admitted with acute necrotizing emphysematous pancreatitis complicated by posterior reversible encephalopathy syndrome. She remained normotensive on presentation and during the hospital stay. All her investigations were within normal except double-stranded DNA, which came positive. This could explain the immune-mediated mechanism leading to endothelial dysfunction. This case has been presented for its rarity. When acute neurological abnormalities occur in the context of systemic inflammatory conditions, such as acute pancreatitis, it is crucial to take PRES into account. Keywords: posterior reversible encephalopathy syndrome, emphysematous necrotizing biliary pancreatitis, PRES, pancreatitis, case report Introduction Posterior reversible encephalopathy syndrome (PRES), a clinical radiological syndrome, is characterized by the abrupt development of neurological symptoms such as headaches, convulsions, altered sensorium, and visual problems (1). Magnetic resonance imaging shows hyperintensity in T2 and hypointense in T1 representing white matter vasogenic edema. This is mostly a symmetric disorder with asymmetric variants. Symmetric lesions are typically located in the posterior cerebral regions. However, PRES is a misnomer as the edema is also seen in watershed zones other than parietal-occipital regions, thalamus, and sometimes in the anterior circulation. Moreover, the syndrome is not always reversible. Life-threatening complications can develop such as transforaminal cerebellar herniation and focal neurologic deficits, especially if prompt treatment is not initiated (2). Various risk factors or etiologies have been identified with PRES such as hypertensive encephalopathy, preeclampsia, eclampsia, use of cytotoxins or immunosuppressants and also in underlying autoimmune conditions (3). Case report A 41-year-old female was admitted to the surgical ward as a case of Severe Acute Emphysematous Necrotizing Biliary Pancreatitis on conservative management. During the course of the hospital stay, she was intubated for 11 days and successfully extubated. 29
  • 2. 30 Nazir Ahmad et al. FIGURE 1 | Initial CT brain showing hypodense changes in the bilateral parieto-occipital regions. FIGURE 2 | MRI DWI showing diffusion restriction in bilateral parieto-occipital region.
  • 3. 10.54646/bijnn.2024.17 31 FIGURE 3 | MRI flair image showing signal change in bilateral parieto-occipital region. On the 56th day, it was noticed during surgical rounds that the patient was not communicating and she was referred to a neurologist. On examination, the patient was conscious, awake, making eye contact, not communicating, not responding to commands. The pupils were 3 mm reacting to light, and the patient was spontaneously moving her eyeballs in all directions. She had no obvious facial asymmetry and was moving all her limbs to pain. Her reflexes were normal and plantar - bilateral flexor. CT brain (Figure 1) and MRI brain (Figures 2, 3) were done, which showed evidence of posterior reversible encephalopathy syndrome. After returning from CT brain, the patient developed 2 episodes of convulsions. She was treated conservatively with anti-epileptics and angioedema measures. Routine blood investigations were normal while autoimmune workup showed positive anti-dsDNA. Blood pressure remained stable throughout the admission. The repeat CT Brain (Figure 4) done after almost 20 days showed a regressive course of PRES. The patient symptomatically improved and was discharged. Discussion The pathophysiology behind PRES is unknown. Various explanations have been proposed like cerebral vasoconstriction resulting in brain infarcts, cerebral auto- regulation failure with vasogenic edema, and endothelial injury with blood–brain barrier disturbance resulting in vasogenic edema. This vasogenic theory explains that development of PRES in cases of accelerated hypertension. The posterior circulation of the brain is comparably less innervated with sympathetic nerves, which is most likely the cause of PRES’s preferential involvement of the posterior portion of the brain (4, 5). At least 20% of patients with PRES have normal blood pressure throughout the course of the disease. Endothelial dysfunction in these patients is probably caused by immune- related mechanisms, such as the secretion of inflammatory cytokines like interleukin 1, tumor necrosis factor-1, and interferon factor gamma.(5) To the extent of for knowledge, very few cases of PRES associated with pancreatitis have been reported. Murphy et al. and Magno have presented reports of PRES associated
  • 4. 32 Nazir Ahmad et al. FIGURE 4 | Follow-up CT brain showing regression of previous signal changes. with pancreatitis due to alcoholism (6, 7). Palmer et al. have presented a case report of PRES associated with pancreatic carcinoma (8). Posterior reversible encephalopathy syndrome should be taken into consideration when a patient with acute pancreatitis experiences headache, visual impairment, and seizures. Acute pancreatitis may cause cytotoxic and vasogenic edema by damaging secondary cerebral vascular endothelium through both local and systemic inflammatory pathway activation (3). Our patient presented with Severe Acute Emphysematous Necrotizing Biliary Pancreatitis leading to PRES. She remained normotensive on presentation and during the hospital stay. All her investigations were within normal except double-stranded DNA, which came positive. This could explain the immune-mediated mechanism leading to endothelial dysfunction. Conclusion This case has been presented for its rarity. When acute neurological abnormalities occur in the context of systemic inflammatory conditions, such as acute pancreatitis, it is crucial to take PRES into account. To the extent of our knowledge, this is the first case with emphysematous necrotizing pancreatitis leading to PRES with complete recovery. Conflict of interest The authors declare that research was conducted in the absence of any commercial or financial relationships that could be construed as potential conflict of interest. Ethics statement As per Emirates health service policy, ethical committee approval is not needed for case reports. Author contributions MA: study design and conceptual development. AD: study design and conceptual development. SI: data acquisition and analysis. DK: conceptual development.
  • 5. 10.54646/bijnn.2024.17 33 All authors contributed to the article and approved the submitted version. Consent for publication Written consent for publication is taken from the patient. References 1. Hobson E, Craven I, Blank S. Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness. Perit Dial Int. (2012) 32:590–4. doi: 10.3747/pdi.2012. 00152 2. Zelaya J, Al-Khoury L. Posterior reversible encephalopathy syndrome. StatPearls. Treasure Island (FL): StatPearls Publishing (2023). 3. Luo J, Zhan Y, Hu Z. Posterior reversible encephalopathy syndrome in a woman with pancreatitis. Chin Med J. (2019) 132:2265–7. doi: 10.1097/ CM9.0000000000000431 4. Choe S, Ganta N, Alnabwani D, Hechter S, Alsaoudi G, Patel V, et al. A case report of severe posterior reversible encephalopathy syndrome due to accelerated hypertension in a young patient. Cureus. (2022) 14:e26918. doi: 10.7759/cureus.26918 5. Bonavia L, Jackson J, Jurevics R. Posterior reversible encephalopathy syndrome in acute pancreatitis: a rare stroke mimic. BMJ Case Rep. (2020) 13:e232228. doi: 10.1136/bcr-2019-232228 6. Murphy T, Al-Sharief K, Sethi V, Ranger G. Posterior reversible encephalopathy syndrome (PRES) after acute pancreatitis. West J Emerg Med. (2015) 16:1173–4. doi: 10.5811/westjem.2015.8.28347 7. Magno Pereira V, Marote Correia L, Rodrigues T, Serrão Faria G. Acute pancreatitis and posterior reversible encephalopathy syndrome: a case report. Acta Med Port. (2016) 29:567–71. doi: 10.20344/amp.7368 8. Palmer K, Weerasuriya S, Whitelaw B, Srirajaskanthan R. Posterior reversible encephalopathy syndrome in a patient with a metastatic pancreatic neuroendocrine tumour and ectopic Cushing’s syndrome. Endocrinol Diabetes Metab Case Rep. (2021) 2021:21–14. doi: 10.1530/ EDM-21-0014