3rd APLAR Grand Round Blackboard

Question 1

A 28 year old female diagnosed with SLE with proliferative lupus nephritis being treated with pulse cyclophosphamide and high dose prednisolone presented with acute hypertension (BP 180/120 mmHg), headache and visual disturbance. Her initial brain imaging (MRI) showed prominent cortical and subcortical white matter signal hyperintensity on T2 FLAIR involving bilateral occipital and left anterior parietal lobe and patchy hyperintense T2 FLAIR signal foci within the right cerebellar hemisphere. Given her current condition, which of the following statement is FALSE?

Your Answer is Incorrect

Answer with Detailed explanation

The correct answer is C. A lumbar puncture is required to evaluate all the patients with suspected PRES.

A lumbar puncture is not required for the evaluation of most patients with suspected PRES but may be obtained if there is a specific concern for meningitis, encephalitis, or malignancy. In PRES, cerebrospinal fluid (CSF) typically shows a modestly elevated protein level (mean 58 mg/dL in one study) but no pleocytosis. An elevated white blood cell count in the CSF should prompt consideration of other diagnoses. In malignant hypertension, the initial aim of treatment is to lower the diastolic pressure to approximately 100 to 105 mmHg. This goal should be achieved within two to six hours, with the maximum initial fall in blood pressure not exceeding 25 percent of the presenting value. More aggressive blood pressure lowering is generally unnecessary and may reduce the blood pressure below the autoregulatory range, possibly leading to cerebral ischemia and increasing the risk of coronary and renal ischemia. PRES is usually reversible. The clinical and radiological findings generally resolve within a few weeks. Diffusion-weighted imaging (DWI) aids in the distinction of PRES from a top-of-the-basilar stroke. The vasogenic edema characteristic of PRES is usually visualised as a hypo- or isointense signal on DWI and increased signal on apparent diffusion coefficient (ADC) maps. By contrast, acute cerebral infarction produces marked hyperintensity on DWI and hypointensity on ADC maps.

Reference

1. Datar S, Singh TD, Fugate JE, Mandrekar J, Rabinstein AA, Hocker S. Albuminocytologic Dissociation in Posterior Reversible Encephalopathy Syndrome. Mayo Clin Proc. 2015 Oct;90(10):1366-71. doi: 10.1016/j.mayocp.2015.07.018. Epub 2015 Sep 5. PMID: 26349950.
2. Triplett JD, Kutlubaev MA, Kermode AG, Hardy T. Posterior reversible encephalopathy syndrome (PRES): diagnosis and management. Pract Neurol. 2022 Jun;22(3):183-189. doi: 10.1136/practneurol-2021-003194. Epub 2022 Jan 19. PMID: 35046115.
3. Raman, Rajesh et al. “Various Imaging Manifestations of Posterior Reversible Encephalopathy Syndrome (PRES) on Magnetic Resonance Imaging (MRI).” Polish journal of radiology vol. 82 64-70. 7 Feb. 2017, doi:10.12659/PJR.899960
4. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol. 2002 Jun-Jul;23(6):1038-48. PMID: 12063238; PMCID: PMC7976914.

By Dr Shweta Nakarmi, Consultant Rheumatologist, National Center for Rheumatic Diseases, Kathmandu, Nepal

Your Answer is Correct

Answer with Detailed explanation

A lumbar puncture is not required for the evaluation of most patients with suspected PRES but may be obtained if there is a specific concern for meningitis, encephalitis, or malignancy. In PRES, cerebrospinal fluid (CSF) typically shows a modestly elevated protein level (mean 58 mg/dL in one study) but no pleocytosis. An elevated white blood cell count in the CSF should prompt consideration of other diagnoses. In malignant hypertension, the initial aim of treatment is to lower the diastolic pressure to approximately 100 to 105 mmHg. This goal should be achieved within two to six hours, with the maximum initial fall in blood pressure not exceeding 25 percent of the presenting value. More aggressive blood pressure lowering is generally unnecessary and may reduce the blood pressure below the autoregulatory range, possibly leading to cerebral ischemia and increasing the risk of coronary and renal ischemia. PRES is usually reversible. The clinical and radiological findings generally resolve within a few weeks. Diffusion-weighted imaging (DWI) aids in the distinction of PRES from a top-of-the-basilar stroke. The vasogenic edema characteristic of PRES is usually visualised as a hypo- or isointense signal on DWI and increased signal on apparent diffusion coefficient (ADC) maps. By contrast, acute cerebral infarction produces marked hyperintensity on DWI and hypointensity on ADC maps.

Reference

1. Datar S, Singh TD, Fugate JE, Mandrekar J, Rabinstein AA, Hocker S. Albuminocytologic Dissociation in Posterior Reversible Encephalopathy Syndrome. Mayo Clin Proc. 2015 Oct;90(10):1366-71. doi: 10.1016/j.mayocp.2015.07.018. Epub 2015 Sep 5. PMID: 26349950.
2. Triplett JD, Kutlubaev MA, Kermode AG, Hardy T. Posterior reversible encephalopathy syndrome (PRES): diagnosis and management. Pract Neurol. 2022 Jun;22(3):183-189. doi: 10.1136/practneurol-2021-003194. Epub 2022 Jan 19. PMID: 35046115.
3. Raman, Rajesh et al. “Various Imaging Manifestations of Posterior Reversible Encephalopathy Syndrome (PRES) on Magnetic Resonance Imaging (MRI).” Polish journal of radiology vol. 82 64-70. 7 Feb. 2017, doi:10.12659/PJR.899960
4. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol. 2002 Jun-Jul;23(6):1038-48. PMID: 12063238; PMCID: PMC7976914.

By Dr Shweta Nakarmi, Consultant Rheumatologist, National Center for Rheumatic Diseases, Kathmandu, Nepal

Question 2

Which of the following MRI finding is less likely to be seen in PRES?

Your Answer is Incorrect

Answer with Detailed explanation

The correct answer is B. Unilateral involvement

The findings in PRES are usually bilaterally symmetrical.

The most commonly described abnormality in PRES consists of symmetrical cortical and subcortical hyperintense signals on T2 and FLAIR-weighted MR images in the parieto-occipital lobes of both hemispheres. Similar areas of altered signal intensity can also be seen in other locations such as the frontal lobes, cerebellum, brainstem and basal ganglia. Petechial and extensive parenchymal haemorrhages may be seen in up to 20% of the patients with PRES. This complication is more common in patients with coagulopathy or thrombocytopenia. Diffusion-weighted imaging (DWI) aids in the distinction of PRES from a top-of-the-basilar stroke. The vasogenic edema characteristic of PRES is usually visualised as a hypo- or isointense signal on DWI and increased signal on apparent diffusion coefficient (ADC) maps.

Reference

1. Raman, Rajesh et al. “Various Imaging Manifestations of Posterior Reversible Encephalopathy Syndrome (PRES) on Magnetic Resonance Imaging (MRI).” Polish journal of radiology vol. 82 64-70. 7 Feb. 2017, doi:10.12659/PJR.899960
2. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol. 2002 Jun-Jul;23(6):1038-48. PMID: 12063238; PMCID: PMC7976914.
3. Lai CC, Chen WS, Chang YS, Wang SH, Huang CJ, Guo WY, Yang WC, Huang DF. Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2013 Nov;65(11):1766-74. doi: 10.1002/acr.22047. PMID: 23687067.

By Dr Shweta Nakarmi, Consultant Rheumatologist, National Center for Rheumatic Diseases, Kathmandu, Nepal

Your Answer is Correct

Answer with Detailed explanation

The findings in PRES are usually bilaterally symmetrical. The most commonly described abnormality in PRES consists of symmetrical cortical and subcortical hyperintense signals on T2 and FLAIR-weighted MR images in the parieto-occipital lobes of both hemispheres. Similar areas of altered signal intensity can also be seen in other locations such as the frontal lobes, cerebellum, brainstem and basal ganglia. Petechial and extensive parenchymal haemorrhages may be seen in up to 20% of the patients with PRES. This complication is more common in patients with coagulopathy or thrombocytopenia. Diffusion-weighted imaging (DWI) aids in the distinction of PRES from a top-of-the-basilar stroke. The vasogenic edema characteristic of PRES is usually visualised as a hypo- or isointense signal on DWI and increased signal on apparent diffusion coefficient (ADC) maps.

Reference

1. Raman, Rajesh et al. “Various Imaging Manifestations of Posterior Reversible Encephalopathy Syndrome (PRES) on Magnetic Resonance Imaging (MRI).” Polish journal of radiology vol. 82 64-70. 7 Feb. 2017, doi:10.12659/PJR.899960
2. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol. 2002 Jun-Jul;23(6):1038-48. PMID: 12063238; PMCID: PMC7976914.
3. Lai CC, Chen WS, Chang YS, Wang SH, Huang CJ, Guo WY, Yang WC, Huang DF. Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2013 Nov;65(11):1766-74. doi: 10.1002/acr.22047. PMID: 23687067.

By Dr Shweta Nakarmi, Consultant Rheumatologist, National Center for Rheumatic Diseases, Kathmandu, Nepal