Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: a case report

Abstract

BackgroundPosterior reversible encephalopathy syndrome is a presentation which is diagnosed clinico-radiologically. The primary aetiological processes leading to posterior reversible encephalopathy syndrome are many, which include autoimmune conditions. Polyarteritis nodosa as an aetiological factor for posterior reversible encephalopathy syndrome is rare. We present a case of polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome.Case presentationA 26-year-old South-Asian female presented with left sided focal seizures with secondary generalization and visual disturbance for 2 days duration. She had a prior history of arthralgia and weight loss with no medically explainable cause for young onset hypertension. Examination revealed a right claw hand with a palpable vasculitic type of rash involving both the palmar surfaces. Symptoms responded to management with anti-hypertensives and anti-epileptics. Whole blood count, iron studies, erythrocyte sedimentation rate and C-reactive protein values portrayed an ongoing chronic inflammatory process. Serological studies such as Anti-nuclear antibody, Anti -double stranded deoxyribonucleic acid, Anti-neutrophil cytoplasmic antibody and Anti-cyclic citrulinated peptide were negative. Magnetic resonance imaging revealed high signal intensity on T2 in both occipital lobes. Skin biopsy of the palm revealed moderate vessel vasculitis. Renal imaging revealed structurally abnormal kidneys with micro aneurysms in the right renal vasculature. Repeat magnetic resonance imaging of the brain two months later showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made.ConclusionsPosterior reversible encephalopathy syndrome should not be missed. Investigations for an aetio-pathological cause should be considered including the rarer associations like polyarteritis nodosa.

Polyarteritis nodosa presenting with posterior reversible leukoencephalopathy syndrome

“…The distribution of signal abnormalities seen in our case were consistent with the two primary patterns described in PRES 17 18. Navinan et al reported a case of PAN-associated PRES in a woman in her 20s presenting with seizures and visual disturbance on a background of known young-onset hypertension 14. Further case reports by Arai et al 15 and Stanzani et al 16 propose PAN-associated PRES as the underlying the aetiology in patients presenting with PRES.…”

“…The most frequently described CNS manifestation is a diffuse encephalopathy 6. PRES is associated with various autoimmune diseases, including PAN, which has been previously described in a small number of cases 13–16. The distribution of signal abnormalities seen in our case were consistent with the two primary patterns described in PRES 17 18.…”

Widening the Neuroimaging Features of Adenosine Deaminase 2 Deficiency

“…PRES has been occasionally reported in patients with DADA2, 11,14 as well as in patients with PAN with unknown ADA2 status. 22 Both our cases occurred during childhood and manifested with seizures associated with typical neuroimaging patterns. 23 These events occurred in the context of arterial hypertension, a complication of DADA2 6 and a wellknown risk factor for PRES 23 ; none of the patients was under anti-TNF treatment.…”

Malignant Posterior Reversible Encephalopathy Syndrome—An Exacting Challenge for Neurocritical Care Physicians

“…Posterior reversible encephalopathy syndrome is a clinicoradiological syndrome, described in 1996 by Hinchey et al in a series of case reports as a “reversible posterior leukoencephalopathy syndrome” 2 seen in association with clinical features of headache, focal neurological deficits, vomiting, seizures, usually generalized tonic-clonic, 3 and visual defects such as cortical blindness, homonymous hemianopia, visual hallucinations, 3,4 along with the hallmark radiological signs of subcortical white matter lesions on MRI, most noticeable in the parietal and occipital lobes, in many cases bilateral such as in this particular case as seen in Figure 2 . 5 The clinical features in collusion with the classic MRI findings lead to a diagnosis of PRES in most of cases; thus, further investigations are rarely required, except to find out the causative factors of PRES.…”