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However, because they can sometimes be caused by problems at the level of the cerebral cortex, attention must be paid to the presence of other associated neurological symptoms.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">In this paper we describe the case of a 73-year-old man, without a remarkable previous medical history, who presented to our clinic with a two-week history of blurred vision, metamorphopsia, and a mild balance disorder. A neurological examination revealed normal findings, except for an unstable gait with left-sided deviation. Both ophthalmological and otorhinolaryngological examinations were unremarkable. Laboratory tests and an emergency brain computed tomography (CT) scan showed no alterations either. A brain magnetic resonance imaging (MRI) scan only showed signs of demyelination secondary to a small vessel disease.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The patient’s condition evolved poorly, with a worsening of his metamorphopsia and the onset of visual hallucinations, an inability to fix his gaze, and subsequent blindness. In addition, he developed incoherent speech, disorientation, bidirectional horizontal nystagmus, balance disorder, and predominantly left-sided dysdiadochokinesia. No changes were detected in a follow-up MRI and ophthalmological exam. However, an electroencephalogram revealed evidence compatible with a generalized encephalopathy. A cerebrospinal fluid (CSF) analysis was also performed, observing normal figures and no onconeural antibodies. On the other hand, a positron emission tomography (PET) scan showed signs of severe hypometabolism in the right parietal, occipital, and temporal cerebral cortex. No improvement was achieved with antiepileptics nor high doses of thiamine or corticosteroids. A second electroencephalogram was performed, observing signs of generalized brain involvement, with abundant triphasic waves adopting a pseudoperiodic pattern, a finding compatible with spongiform encephalopathy.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient eventually developed severe cognitive impairment, myoclonus, and dysphagia, and passed away 40 days later. The postmortem examination revealed small-medium vacuolar spongiosis, astrocytosis, and neuronal loss, especially in the primary visual cortex, the thalamus, the cerebellum, and the entorhinal cortex, with scrapie prion protein (PrP<span class="elsevierStyleSup">Sc</span>) deposits, all being findings compatible with sporadic Creutzfeldt-Jakob disease (CJD). A study of the <span class="elsevierStyleItalic">PRNP</span> gene detected the MM variant without mutations, and positivity for the 14-3-3 protein in the CSF was also observed.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Creutzfeldt-Jakob disease is a rapidly progressing spongiform encephalopathy caused by prions and fatal within less than one year in the majority of cases. It is categorized as a type of rapidly progressive dementia, is the most frequent prion disease in humans, and can be classified as familial, acquired, or sporadic. An alteration in human prion protein PrP<span class="elsevierStyleSup">c</span> results in an aberrant protein (PrP<span class="elsevierStyleSup">Sc</span>) that accumulates in the central nervous system in the form of aggregates. Its clinical manifestations are varied, including rapidly progressive dementia, cortical deficits (aphasia and visual disturbances), cerebellar manifestations, extrapyramidal symptoms, myoclonus, and psychiatric alterations. The classic or myoclonic form manifests with progressive cognitive impairment with akinetic mutism and myoclonus in early stages.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The Heidenhain variant is the most aggressive and rapidly progressive form of CJD. Visual alterations and even cortical blindness may occur, often preceding cognitive impairment and myoclonus.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> Visual symptoms in CJD at onset of the Heidenhain variant is rare (3.1%–20% of cases).<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> The most frequent visual manifestations are a lack of object definition, metamorphopsia, color perception disorders (chloropia), and visual hallucinations associated with cortical blindness, potentially together with nystagmus due to the related cerebellar damage.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">An MRI scan might show restricted diffusion and hyperintensity in the fluid-attenuated inversion recovery (FLAIR) sequence in the affected regions, while an electroencephalogram usually reveals diffuse slowing and periodic triphasic waves in the posterior regions, which are highly specific findings for sporadic CJD and the Heidenhain variant. The definitive diagnosis of CJD is reached through a histology study showing spongiform changes, neuronal loss, astrogliosis, and PrP<span class="elsevierStyleSup">Sc</span> protein aggregates, especially in the most affected regions (occipital cortex in the Heidenhain variant).<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The Heidenhain variant is an uncommon form of CJD that can sometimes present with visual symptoms. Hence, this entity should be considered as a potential differential diagnosis when a clinical picture of visual disturbances with metamorphopsia is followed by rapidly progressive cognitive impairment.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Pitarch Fabregat J, García Escrivà A, Pellicer Cabo Á. Alteración visual como manifestación inicial de la enfermedad de Creutzfeldt-Jakob. Med Clin (Barc). 2021;157:e335–e336.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Metamorphopsia: an overlooked visual symptom" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "E. Midena" 1 => "S. 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Steinhoff" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1001/archneur.56.1.55" "Revista" => array:6 [ "tituloSerie" => "Arch Neurol" "fecha" => "1999" "volumen" => "56" "paginaInicial" => "55" "paginaFinal" => "61" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9923761" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015700000011/v1_202112030828/S2387020621006392/v1_202112030828/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015700000011/v1_202112030828/S2387020621006392/v1_202112030828/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621006392?idApp=UINPBA00004N" ]
