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Claw hand and vasculitic rash: Image on the left depicts a palpable vasculitic rash on the palmar surface while the image on the right depicts a right sided claw hand.
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Posterior 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. W...
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Context 1
... general examination, her consciousness level was a Glasgow coma scale (GCS) level of 14/15 with an ele- vated blood pressure of 190/120 mmHg. Palms of her hands revealed a blotchy palpable purpuric type of vaculi- tic rash (Figure 1). Nervous system examination revealed a clawed right hand in isolation without any other deficit or abnormality (Figure 1). ...Similar publications
Cancer and treatments may induce cognitive impairments in cancer patients, and the causal link between chemotherapy and cognitive dysfunctions was recently validated in animal models. New cancer targeted therapies have become widely used, and their impact on brain functions and quality of life needs to be explored. We evaluated the impact of everol...
Posterior reversible encephalopathy syndrome (PRES) is a transient condition characterized by altered mental status, seizure, headache, and visual disturbance with typical neuro-imaging findings in the bilateral parieto-occipital regions. Clinicians should be aware of this syndrome because delayed diagnosis and treatment result in irreversible neur...
Citations
... 6 PRES is associated with various autoimmune diseases, including PAN, which has been previously described in a small number of cases. [13][14][15][16] 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 PANassociated PRES in a woman in her 20s presenting with seizures and visual disturbance on a background of known young-onset hypertension. ...
A man in his 20s presented following a generalised tonic–clonic seizure on a background of a recent diagnosis of hepatitis B (HBV). During admission, he was severely hypertensive and imaging findings confirmed a diagnosis of posterior reversible leukoencephalopathy syndrome (PRES). The patient subsequently developed multiorgan involvement with an axonal sensorimotor neuropathy, vascular cutaneous lesions and multiple bilateral renal and splenic infarcts. Based on the 2012 Revised International Chapel Hill Consensus Criteria, a diagnosis of polyarteritis nodosa (PAN) with secondary PRES was made. The patient was given intravenous methylprednisolone, followed by a prolonged course of oral prednisolone, and tenofovir antiviral therapy to target HBV seroconversion. He made a good neurological recovery with resolution of imaging changes. This case highlights the importance of a low threshold for systemic screening for young patients presenting with PRES secondary to uncontrolled hypertension and the importance of viral screening, particularly for HBV.
... The choice depends on the severity of the condition, the patient's characteristics, and the risk of adverse effects. In patients with mild PAN and evidence of infection with HBV or HCV, we suggest treating most patients initially with antivirals only, rather than also treating initially with immunosuppressive medications [5,9,[11][12][13]. ...
... In the case of Stanzani et al. [11], a 52-year-old woman presented a fatal evolution after 7 months despite treatment with steroids, immunoglobulins, and cyclophosphamide. In the case of Navinan et al. [12], a 26-year-old woman treated with antihypertensives and immunosuppressive therapy presented a favorable evolution. In the French nationwide multicentre study, two cases (57-and 49-year-old women) were found, both treated with immunosuppressive therapy with good response [13,14]. ...
A 21-year-old woman with unremarkable past health presented with an intense headache and visual disturbance, followed by a secondary generalized tonic-clonic focal seizure. She had a history of arthritis, livedo reticularis, myalgia, elevated erythrocyte sedimentation rate, and nonspecific constitutional manifestations (fever and weight loss). Examination revealed new-onset severe hypertension. Complete immunological studies were negative. Electroencephalogram showed abnormalities compatible with secondarily generalized tonic–clonic focal occipital seizures. Brain magnetic resonance imaging (MRI) revealed high signal intensity on T2 in occipital lobes, cerebellum, and brainstem and renal imaging multiple hypoenhancing parenchymal images (renal infarction). Follow-up brain MRI at 3 months showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made.
... 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. ...
SUMMARY: Adenosine deaminase 2 deficiency (OMIM #615688) is an autosomal recessive disorder characterized by a wide clinical
spectrum, including small- and medium-sized vessel vasculopathies, but data focusing on the associated neuroimaging features are
still scarce in the literature. Here, we describe the clinical neuroimaging features of 12 patients with genetically proven adenosine
deaminase 2 deficiency (6 males; median age at disease onset, 1.3 years; median age at genetic diagnosis, 15.5 years). Our findings
expand the neuroimaging phenotype of this condition demonstrating, in addition to multiple, recurrent brain lacunar ischemic and/or
hemorrhagic strokes, spinal infarcts, and intracranial aneurysms, also cerebral microbleeds and a peculiar, likely inflammatory, perivascular
tissue in the basal and peripontine cisterns. Together with early clinical onset, positive family history, inflammatory flares and systemic
abnormalities, these findings should raise the suspicion of adenosine deaminase 2 deficiency, thus prompting genetic evaluation and institution
of tumor necrosis factor inhibitors, with a potential great impact on neurologic outcome.
... Corticosteroid plus a potent immunosuppressive drug, particularly cyclophosphamide (CYC), are the treatment of choice for idiopathic PAN [20]. In some cases, these treatments are not effective, or not well tolerated, thus relapse may occur. ...
... Polyarteritis nodosa is a necrotizing vasculitis that affects small or medium-sized muscular arteries in many different organs [20]. The varying clinical presentations and the rarity of the disease often result in delayed diagnosis [3]. ...
... Peripheral nerves manifestations are seen in 63% of cases, including peripheral neuropathy and mononeuritis multiplex [1]. However, the CNS is less commonly involved [19,20,38]. Constitutional symptoms manifest sub-acutely [39]. ...
Introduction
Polyarteritis Nodosa (PAN) is a rare systemic necrotizing vasculitis and the variety in clinical presentations lead to difficulty in diagnosis. The co-incidence of PAN disease and familial Mediterranean fever (FMF) has been reported and as they share similar clinical symptoms such as fever, abdominal pain, arthralgia, and skin rash the diagnosis could be challenging.
Case report
This report is presented as one of the unusual cases of PAN with intra-parenchymal renal aneurysm rupture, which was misdiagnosed as peri-renal abscess in a patient with FMF.
Case presentation
A 24 year–old Syrian man presented to the Emergency Department of Tishreen Hospital with fever (39 °C), anorexia, general weakness, severe musculoskeletal pain, weight loss and flank tenderness. He had a history of FMF since he was 7 years old and used to be treated with colchicine. Laboratory investigations revealed leukocytosis, anaemia, increased erythrocyte sedimentation rate (99 mm/1st h) and C-reactive protein (CRP) (96 mg/l). Renal function, urine analysis, creatinine clearance, electrolytes and transaminases were normal. Urine and blood culture were negative. The anti-neutrophil cytoplasmic antibody, rheumatoid factor and hepatitis markers were negative. On abdominal ultrasonography, renal parenchyma was hyper-echogenic with surrounding liquid. Antibiotics were provided with no improvement. The patient developed secondary hypertension and MRI brain revealed ischemic cortical and subcortical parietal lesions. PAN was diagnosed based on renal CT angiography showing multiple micro-aneurysms. Consequently, methylprednisolone pulses and cyclophosphamide were introduced with dramatic improvement.
Conclusion
Increased awareness of such clinical association of PAN and FMF and early management may improve the disease outcome.
... The most common manifestation of CNS involvement includes diffuse encephalopathy and a deficit of focal neurological function. Diffuse encephalopathy may present as new onset seizure and headache, reduced level of consciousness or altered vision, among which some of the cases also suffer from reversible encephalopathy syndrome (37)(38)(39). The main manifestations of focal CNS involvement include cerebral infarction (which occurs in 13-17% of PAN patients), hemorrhage, multifocal encephalopathy and episodes of neurological dysfunction that mimic multiple sclerosis (33,40). ...
Primary systemic vasculitis can affect every structure in both the central and peripheral nervous system, causing varied neurological manifestations of neurological dysfunction. Early recognition of the underlying causes of the neurological symptoms can facilitate timely treatment and improve the prognosis. This review highlights the clinical manifestations of primary systemic vasculitis in the nervous system.
... Although hypertension is one of the criteria to diagnose polyarteritis nodosa, posterior reversible encephalopathy syndrome as initial presenting manifestation is rare. [3,4] In a large single-center retrospective study of 69 children seen over 32 years with PAN, only 7 (10%) had the central nervous system involvement with mononeuritis multiplex, peripheral neuropathy, meningitis, stroke, or cranial nerve palsy. [5] In a case series and systematic review of 108 children with posterior reversible encephalopathy syndrome, underlying kidney diseases and malignancies were common causes of this condition. ...
... 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. ...
We report a case of malignant posterior reversible encephalopathy syndrome (PRES) in a 62-year-old Caucasian female with a complex medical history and comorbidities admitted for bowel resection and lysis of iatrogenic bowel adhesions and enterocutaneous fistulas. Postoperatively, the patient developed sudden bilateral visual loss with no other neurologic deficits. Computed tomography scan showed very severe PRES-like changes, confirmed on magnetic resonance imaging (MRI). Systolic blood pressure remained around 170 mm HG. The patient was obtunded and remained unresponsive after MRI, with minimal response and a deteriorating clinical condition. The patient was given hyperosmolar therapy with a mannitol bolus. She recovered well with near resolution of imaging findings.
... Por ejemplo, se ha informado de EPR durante el tratamiento del vasoespasmo en la hemorragia subaracnoidea me diante la terapia triple H, terapia con la que se pre tende lograr una vasodilatación hipertensiva. Sin embargo, ya que en la hemorragia subaracnoidea existe una afectación vascular endotelial y daño de la barrera hematoencefálica, es posible el desarrollo de la EPR por extravasación de los capilares sanguí neos secundaria a la 'inducción' de un flujo san guíneo cerebral aumentado [13,14,1619]. Así, tam bién se ha notificado la EPR en pacientes tratados con fármacos inhibidores del factor de crecimiento vascular endotelial, los cuales suelen inducir HTA además de afectar la permeabilidad vascular y dis minuir la biodisponibilidad del óxido nítrico [20]. En el caso de las enfermedades autoinmunes como el lupus eritematoso sistémico, estos pacientes sue Tabla I. Enfermedades o condiciones asociadas a la encefalopatía posterior reversible (cinco o más casos clínicos publicados). ...
... Hipertensión arterial [1] Infección, sepsis y fallo multiorgánico [2] Trasplante de órganos [3] Enfermedades renales (insuficiencia renal crónica, glomerulonefritis, síndrome nefrótico) [4] Embarazo (eclampsia y mola hidatiforme) [5,6] Lupus eritematoso sistémico [7,8] Vasculitis (enfermedad de Wegener, poliarteritis nudosa, Takayasu, crioglobulinemia) [8][9][10][11][12][13][14][15][16][17][18][19] Quimioterapia inmunosupresora (tacrolimús, ciclosporina, bevacizumab, sunitinib, citarabina, cisplatino, gemcitabina, etc.) [20,21] Neoplasias hematológicas (leucemia, linfoma, mieloma múltiple, síndrome mielodisplásico) [ Púrpura trombótica trombocitopénica [44][45][46][47][48][49][50] Síndrome hemolítico ureico [51][52][53][54][55][56][57] Púrpura de Henoch-Schönlein [58][59][60][61][62] Porfiria [63][64][65][66][67][68] Anemia de células falciformes [69][70][71][72][73][74][75] Trastornos electrolíticos (hipercalcemia, hipomagnesemia) [30, [76][77][78][79][80][81] Síndrome de Guillain-Barré [82][83][84][85][86][87] Drogas simpaticomiméticas (cocaína, fentermina, acido lisérgico, efedrina, fenilpropanolamina, pseudoefedrina) [88][89][90][91][92][93][94] Tumores extra/intraaxiales del tronco cerebral [95][96][97][98][99] La bibliografía de esta tabla aparece como material suplementario en la versión electrónica del artículo (www.neurologia.com). len cursar con afectación renal autoinmune y, de ma nera secundaria, hipoalbuminemia, lo que predispo ne a una afectación en la regulación del flujo sanguí neo cerebral por disfunción vascular endotelial [21]. ...
... El curso de las crisis epilépticas suele ser benigno y, en la mayor parte de los casos, se manifiestan como episodios aislados de corta duración de crisis gene ralizadas, aunque también se han descrito crisis fo cales, especialmente en la edad pediátrica [48]. Sin embargo, no existe una relación entre la afectación cortical radiológica y la manifestación de crisis o estados epilépticos en estos pacientes [14]. ...
Posterior reversible encephalopathy (PRE) is a clinical and radiological entity that is typically characterized by headache, visual disturbances and seizures associated with cortical and subcortical reversible vasogenic edema in neuroimaging.
To present a review of the pathophysiology of this entity, and also the associations of the PRE described in the literature.
Given its clinical presentation, often nonspecific and variable, magnetic resonance imaging is essential for diagnosis. There are a number of well-known triggers, such as hypertensive crisis, eclampsia or certain drugs. The description of increasingly atypical cases from clinical and radiological point of view, and possible new triggers, requires a redefinition of this entity.
The PRE is a set of clinical and radiological manifestations that may not be framed within the word 'syndrome'. Although, the PRE has been reported in some cases irreversible, reversibility concept should be maintained in the definition of this entity, since in most cases the rapid control of the triggering condition allows reversibility of the lesions.
... Posterior reversible encephalopathy syndrome (PRES) is a clinical radiological syndrome which could be induced by pre-eclampsia, transplantation, autoimmune disease (systemic lupus erythematosus, Wegener's granulomatosis, systemic sclerosis), abrupt arterial hypertension (which is probably one of the most important), impaired renal function or drugs (anticalcineurin, chemotherapie) [1][2][3][4][5]. Some atypical cases were also reported in a weightlifter patient after an intensive gym session or as a rare association with a polyarteritis nodosa [6,7]. Renal disease seems to be a risk factor of PRES, as seen in many case reports of PRES in patients, in particular in pediatric population, with acute glomerulonephritis, lupus nephritis or, nephrotic syndrome or small vessel vasculitis [8][9][10]. ...
Posterior reversible encephalopathy syndrome is a clinical radiological syndrome, characterized by acute headache, altered consciousness, seizures and hypertension. The most frequent causes are hypertensive encephalopathy, eclampsia and some immunosuppressive therapies. Here, we describe a 75-year-old man with high blood pressure and anti-neutrophil cytoplasmic antibody associated vasculitis with crescentic glomerulonephritis who was treated with cyclophosphamide bolus and corticoids. Symptoms of posterior reversible encephalopathy syndrome have appeared during a hypertensive crisis, 3 days after cyclophosphamide infusion. Cyclophosphamide was stopped and rituximab therapy introduced. The patient recovered promptly. There are only a few reports of posterior reversible encephalopathy syndrome where cyclophosphamide is the only one culprit and they all concern patients with renal disease.
