Letter to the Editor
Neuroimaging abnormalities in a patient with posterior reversible encephalopathy syndrome due to acute intermittent porphyria
Cambios en la neuroimagen en paciente con síndrome de encefalopatía posterior reversible por porfiria aguda intermitente
E. Rivero Sanz
, J.L. Camacho Velásquez, S. Santos Lasaosa, C. Tejero Juste
Corresponding author
Servicio de Neurología, Hospital Clínico Lozano Blesa, Zaragoza, Spain
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Rivero Sanz, J.L. Camacho Velásquez, S. Santos Lasaosa, C. Tejero Juste" "autores" => array:4 [ 0 => array:4 [ "nombre" => "E." "apellidos" => "Rivero Sanz" "email" => array:1 [ 0 => "ella789@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "J.L." "apellidos" => "Camacho Velásquez" ] 2 => array:2 [ "nombre" => "S." "apellidos" => "Santos Lasaosa" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "Tejero Juste" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Neurología, Hospital Clínico Lozano Blesa, Zaragoza, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Cambios en la neuroimagen en paciente con síndrome de encefalopatía posterior reversible por porfiria aguda intermitente" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 563 "Ancho" => 995 "Tamanyo" => 83642 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">MRI T2-weighted sequences revealing hyperintense lesions in the frontal cortex bilaterally which were more pronounced on the left side.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Acute intermittent porphyria (AIP) is one of the 4 types of acute porphyria presenting with neurological or psychiatric symptoms. In young adults with no specific history, the disease can manifest as encephalopathy associated with neuroimaging abnormalities that may be interpreted in numerous ways. Typical MRI findings resemble those seen in posterior reversible encephalopathy syndrome (PRES), which resolve with medical treatment. We describe the case of a young patient with AIP presenting as epilepsy and MRI alterations compatible with PRES.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Our patient was a 23-year-old woman with no relevant medical history who experienced a loss of consciousness followed by 2 tonic–clonic seizures that resolved after treatment with midazolam. Upon arriving at the emergency department, she underwent a CT scan and laboratory tests, which yielded normal results except for mild hyponatraemia. She was discharged after a 24-hour observation period and instructed to take levetiracetam. One day later, she returned to our hospital due to another seizure and symptoms of disorientation and agitation; she was treated with haloperidol. She was admitted to the neurology department for a thorough study. Basic biochemical, blood, and serological tests, blood cultures, autoimmune studies, and a lumbar puncture all showed normal results, including normal coagulation and iron metabolism, and normal levels of vitamin B<span class="elsevierStyleInf">12</span> and folic acid. However, our patient continued to experience episodes of disorientation, agitation, and fluctuations in level of consciousness, which coincided on one occasion with severe hyponatraemia (114<span class="elsevierStyleHsp" style=""></span>mEq/L). A brain MRI revealed bilateral cortical and subcortical frontal lesions, which were hypointense on T1-weighted sequences, hyperintense on T2-weighted sequences (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), and hyperintense on apparent diffusion coefficient sequences.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Given the patient's clinical symptoms, hyponatraemia, and neuroimaging findings, we suspected viral/autoimmune encephalitis and AIP. The diagnosis of AIP was confirmed when a urine test revealed increased levels of total porphyrin (257<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>hours [normal range: 25-220<span class="elsevierStyleHsp" style=""></span>μg/24<span class="elsevierStyleHsp" style=""></span>hours]), uroporphyrin (200<span class="elsevierStyleHsp" style=""></span>μg/dL [normal values<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>2<span class="elsevierStyleHsp" style=""></span>μg/dL]), porphobilinogen (PBG) (216<span class="elsevierStyleHsp" style=""></span>mg/24<span class="elsevierStyleHsp" style=""></span>hours [normal values<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>2.5<span class="elsevierStyleHsp" style=""></span>mg/24<span class="elsevierStyleHsp" style=""></span>hours]), and delta-aminolevulinic acid (delta-ALA) (105.2<span class="elsevierStyleHsp" style=""></span>mg/24<span class="elsevierStyleHsp" style=""></span>hours [normal range, 0-3.4<span class="elsevierStyleHsp" style=""></span>mg/24<span class="elsevierStyleHsp" style=""></span>hours]). We studied the HMBS gene by direct sequencing and detected 2 heterozygous variants: c.83G>T (p.S28I) and c.825+7G>T (IVS12+7G>T).</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was treated with beta-blockers and a carbohydrate-rich diet; her alterations of consciousness, disorientation, and agitation resolved, leaving no sequelae. In a follow-up consultation 2 months later, she was stable and adhering well to her new diet; MRI showed that lesions had disappeared (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Porphyrias are a group of rare, heritable diseases which interrupt normal synthesis of the haem group in haemoglobin. AIP is the most common of all acute porphyrias, a group that includes hereditary coproporphyria (HC), porphyria variegata (PV), and delta-aminolevulinic acid dehydratase porphyria (ALAD-P).<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">1</span></a> Acute porphyria may manifest with severe episodes that may be triggered by infections, alcohol, drugs, or stress, among other factors. Both men and women can inherit AIP; however, the disease is 5 times more likely to be expressed in women, especially during the third decade of life.<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">1,2</span></a> Neurological symptoms are common and may be fatal in 1% of the cases. Autonomic dysfunction manifests as abdominal pain, nausea, vomiting, diarrhoea, hypertension, and tachycardia. Peripheral nervous system involvement may cause paraesthesia, generalised weakness, and respiratory paralysis. Agitation, disorientation, anxiety, and seizures result from central nervous system involvement.<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">3,4</span></a> Patients with AIP frequently show electrolyte alterations, the most common being hyponatraemia, which occurs in 40% of the cases. This is thought to be due to gastrointestinal sodium loss, fluid overload, and reduced secretion of antidiuretic hormone.<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Acute porphyria is difficult to diagnose since symptoms are highly variable and non-specific. If it is suspected, a urine test must be conducted during the episode. Several theories have been proposed to explain the mechanism of neurotoxicity in AIP, including accumulation of haem precursors in neurons, which would result in dysfunction of the Na<span class="elsevierStyleSup">+</span>/K<span class="elsevierStyleSup">+</span> pump, altering membrane potentials and leading to neuronal cell death and axonal degeneration.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In acute porphyria, ALA and PGB levels in urine tests are more than 10 times higher than normal, as seen in our patient.<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">7,8</span></a> Levels of these 2 components are high in HC and PV, although these increases are less marked and long-lasting than in AIP. Increased ALA levels can also be found in other metabolic diseases, including lead poisoning and ALAD-P.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">8</span></a> In the latter, total porphyrin levels in urine are not elevated, which helps differentiate ALAD-P from the other 3 types of acute porphyria. Coproporphyrin levels are very high in HC; this diagnosis was ruled out in our patient's case since she displayed normal levels of coproporphyrin. In PV and HC, symptoms are usually neuropsychiatric and cutaneous, whereas in our patient the disease initially manifested as seizures, chronic abdominal pain, paraesthesia, hypertension, and no skin lesions. These clinical findings, together with the results from the urine analysis, are more indicative of AIP.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Genetic testing is considered the gold standard diagnostic tool for acute porphyrias. The mutations in our patient were uncertain since we identified 2 previously undetected variants. The first of these (c.83G>T) significantly alters its protein and may therefore be the cause of the disease, whereas the second (c.825+7G>T) seems to be a polymorphism illustrating the heterogeneity of the mutations that cause AIP.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Neuroimaging studies are also useful for the differential diagnosis of AIP. The most frequent manifestation of the disease is PRES,<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">5</span></a> a clinical and radiological entity with characteristic features on CT and MR images of the central nervous system.<a class="elsevierStyleCrossRefs" href="#bib0100"><span class="elsevierStyleSup">2,4,7</span></a> Typical neuroimaging findings in PRES include cortical vasogenic oedema. However, despite its name, these lesions are rarely located exclusively in the posterior area of parieto-occipital white matter; rather, the frontal lobes, basal ganglia, and cerebellum are more commonly affected.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">8</span></a> In a recently published study of FLAIR and DWI sequences in PRES, most of the patient sample (54%) displayed exclusively frontal involvement,<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">8</span></a> in another study, 77% of the patients with frontal involvement also showed parieto-occipital involvement.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">9</span></a> In MRI, lesions are iso- or hypointense on T1 and hyperintense on T2.<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">10,11</span></a> Our patient displayed hypointensities on T1 and hyperintensities on T2 in both frontal lobes (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>). DWI MRI is also useful since it helps differentiate between oedema secondary to a cytotoxic process (as in stroke) and vasogenic oedema.<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">12,13</span></a> Diffusion-weighted MR images reveal increased signal intensity in cytotoxic oedema, whereas in vasogenic oedema (occurring in PRES) MRI shows iso- or hypointense lesions.<a class="elsevierStyleCrossRefs" href="#bib0160"><span class="elsevierStyleSup">14,15</span></a> In the context of intense or prolonged seizures, the literature describes MRI changes in the form of hyperintensities in cortico-subcortical areas on T2, which points to fluid overload. These lesions show restricted diffusion in diffusion sequences, which is more typical of cytotoxic oedema than of vasogenic oedema.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">14</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The pathophysiology of PRES is not well known. The vascular hypothesis postulates that peripheral vasospasm occurs during an attack of porphyria and causes brain manifestations resembling those of malignant hypertension.<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">15,16</span></a> Other possible mechanisms may be the neurotoxic effects of the increase in porphyrins and their precursors, or a deficit of cofactors in the synthesis of the haem group.<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">16</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The differential diagnosis of PRES includes acute ischaemic stroke, cerebral venous thrombosis, pontine encephalopathy in the context of mitochondrial encephalomyopathy (MELAS), Creutzfeldt-Jakob disease, and cerebral gliomatosis.<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">17,18</span></a> These entities can be ruled out based on the patient's clinical history, examination, and laboratory and neuroimaging findings.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The case presented here proves once again that AIP can have an insidious onset with common and highly variable neurological symptoms. MRI is a useful diagnostic tool for acute porphyrias typically manifesting with PRES, whose symptoms will remit when properly treated.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors have received no private or public funding for this case report.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Funding" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflict of interest" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Rivero Sanz E, Camacho Velásquez JL, Santos Lasaosa S, Tejero Juste C. Cambios en la neuroimagen en paciente con síndrome de encefalopatía posterior reversible por porfiria aguda intermitente. Neurología. 2016;31:580–583.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 964 "Ancho" => 1300 "Tamanyo" => 122171 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">MRI gadolinium-enhanced T1-weighted sequences showing a hypointense lesion in the left frontal cortex with no contrast uptake.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 563 "Ancho" => 995 "Tamanyo" => 83642 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">MRI T2-weighted sequences revealing hyperintense lesions in the frontal cortex bilaterally which were more pronounced on the left side.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:18 [ 0 => array:3 [ "identificador" => "bib0095" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Porphyrias at a glance: diagnosis and treatment" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "M.D. Cappellini" 1 => "V. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 4 | 0 | 4 |
| 2024 October | 49 | 4 | 53 |
| 2024 September | 44 | 4 | 48 |
| 2024 August | 43 | 2 | 45 |
| 2024 July | 46 | 5 | 51 |
| 2024 June | 51 | 6 | 57 |
| 2024 May | 35 | 2 | 37 |
| 2024 April | 50 | 4 | 54 |
| 2024 March | 50 | 9 | 59 |
| 2024 February | 65 | 20 | 85 |
| 2024 January | 72 | 9 | 81 |
| 2023 December | 62 | 3 | 65 |
| 2023 November | 81 | 4 | 85 |
| 2023 October | 78 | 5 | 83 |
| 2023 September | 34 | 8 | 42 |
| 2023 August | 58 | 2 | 60 |
| 2023 July | 54 | 9 | 63 |
| 2023 June | 67 | 8 | 75 |
| 2023 May | 126 | 7 | 133 |
| 2023 April | 82 | 12 | 94 |
| 2023 March | 67 | 7 | 74 |
| 2023 February | 44 | 6 | 50 |
| 2023 January | 61 | 0 | 61 |
| 2022 December | 39 | 6 | 45 |
| 2022 November | 82 | 6 | 88 |
| 2022 October | 90 | 7 | 97 |
| 2022 September | 88 | 21 | 109 |
| 2022 August | 44 | 11 | 55 |
| 2022 July | 26 | 12 | 38 |
| 2022 June | 42 | 10 | 52 |
| 2022 May | 32 | 17 | 49 |
| 2022 April | 44 | 10 | 54 |
| 2022 March | 58 | 12 | 70 |
| 2022 February | 70 | 8 | 78 |
| 2022 January | 48 | 6 | 54 |
| 2021 December | 47 | 8 | 55 |
| 2021 November | 42 | 8 | 50 |
| 2021 October | 44 | 7 | 51 |
| 2021 September | 44 | 13 | 57 |
| 2021 August | 47 | 11 | 58 |
| 2021 July | 31 | 5 | 36 |
| 2021 June | 33 | 15 | 48 |
| 2021 May | 72 | 10 | 82 |
| 2021 April | 150 | 31 | 181 |
| 2021 March | 67 | 14 | 81 |
| 2021 February | 60 | 8 | 68 |
| 2021 January | 65 | 16 | 81 |
| 2020 December | 68 | 8 | 76 |
| 2020 November | 60 | 9 | 69 |
| 2020 October | 41 | 9 | 50 |
| 2020 September | 31 | 8 | 39 |
| 2020 August | 48 | 8 | 56 |
| 2020 July | 41 | 8 | 49 |
| 2020 June | 33 | 13 | 46 |
| 2020 May | 31 | 10 | 41 |
| 2020 April | 45 | 5 | 50 |
| 2020 March | 45 | 3 | 48 |
| 2020 February | 59 | 6 | 65 |
| 2020 January | 60 | 16 | 76 |
| 2019 December | 59 | 16 | 75 |
| 2019 November | 28 | 9 | 37 |
| 2019 October | 54 | 12 | 66 |
| 2019 September | 92 | 5 | 97 |
| 2019 August | 68 | 2 | 70 |
| 2019 July | 31 | 10 | 41 |
| 2019 June | 71 | 39 | 110 |
| 2019 May | 193 | 44 | 237 |
| 2019 April | 89 | 22 | 111 |
| 2019 March | 12 | 12 | 24 |
| 2019 February | 20 | 5 | 25 |
| 2019 January | 23 | 7 | 30 |
| 2018 December | 10 | 5 | 15 |
| 2018 November | 24 | 4 | 28 |
| 2018 October | 27 | 6 | 33 |
| 2018 September | 23 | 2 | 25 |
| 2018 August | 4 | 6 | 10 |
| 2018 July | 18 | 4 | 22 |
| 2018 June | 7 | 2 | 9 |
| 2018 May | 2 | 6 | 8 |
| 2018 April | 4 | 2 | 6 |
| 2018 March | 5 | 1 | 6 |
| 2018 February | 6 | 2 | 8 |
| 2018 January | 9 | 5 | 14 |
| 2017 December | 9 | 1 | 10 |
| 2017 November | 8 | 3 | 11 |
| 2017 October | 9 | 6 | 15 |
| 2017 September | 11 | 5 | 16 |
| 2017 August | 21 | 5 | 26 |
| 2017 July | 15 | 4 | 19 |
| 2017 June | 14 | 3 | 17 |
| 2017 May | 15 | 3 | 18 |
| 2017 April | 12 | 2 | 14 |
| 2017 March | 6 | 20 | 26 |
| 2017 February | 8 | 5 | 13 |
| 2017 January | 7 | 3 | 10 |
| 2016 December | 30 | 7 | 37 |
| 2016 November | 33 | 7 | 40 |
| 2016 October | 43 | 14 | 57 |
| 2016 September | 11 | 7 | 18 |
| 2016 August | 1 | 5 | 6 |
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