Letter to the Editor
Hashimoto's encephalopathy associated with primary biliary cirrhosis and vitiligo
Encefalitis de Hashimoto asociada a cirrosis biliar primaria y vitíligo
Álvaro Peña-Irún
Medicina Interna, Centro de Salud El Sardinero, Santander, Cantabria, Spain
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This disease has gained importance within the differential diagnosis of encephalopathy of unknown origin, given its autoimmune and reversible nature, with good response to glucocorticoid treatment.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 74-year-old patient, whose history included vitiligo and primary biliary cirrhosis diagnosed 9 years earlier and treated with ursodeoxycholic acid. Previously autonomous and maintaining all their intellectual functions, the patient began to experience changes in their personality, apraxia when getting dressed and a fluctuating ability to recognise family members. After 2 months the patient had severe cognitive impairment with mutism, unsteady gait and loss of bowel control. The constants were normal and they showed disorientation, inability to name objects, incoherent speech and emotional lability. Examination revealed generalised hyperreflexia with clonus in the right foot. The analytical tests for blood count, biochemistry with vitamin B<span class="elsevierStyleInf">12</span>, folic acid and thyroid function were normal. Tumour markers and serum protein were within normal limits. Celiac disease serology, HIV and syphilis tests were negative. Cerebrospinal fluid (CSF) showed an increase of proteins with few lymphocytes and an absence of oligoclonal bands. Normal chest radiograph. The immunological study highlighted the already known positive results of antinuclear antibodies and antimitochondrial antibodies, and the high thyroid anti-peroxidase (TPO) antibodies (196<span class="elsevierStyleHsp" style=""></span>IU/ml), the anti-SSA and SSB, anti-DNA, anticytoplasm of the neutrophil antibodies and antineuronal antibodies being negative. On the other hand, the electroencephalogram (EEG) showed findings consistent with diffuse encephalopathy of a moderate severity, and cranial magnetic resonance imaging (MRI), unspecified abnormalities of white matter. Suspecting Hashimoto's encephalitis, treatment was begun with prednisone at 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day with good response and negativisation of anti-TPO antibodies.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Hashimoto's encephalitis is a neurological syndrome associated with high titers of antithyroid antibodies once other diagnostic alternatives have been discarded,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> and which responds well to corticosteroids or other immunosuppressants. It is a rare disease with an estimated prevalence of 2/10<span class="elsevierStyleSup">5</span> inhabitants, and few documented cases. It manifests itself with a variety of neuropsychiatric symptoms and two common patterns of presentation: subacute progressive cognitive impairment and “stroke-like” manifestations.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> Diagnosis is based on clinical analysis, presence of antithyroid antibodies and response to glucocorticoid treatment. We can base ourselves on the analysis of the CSF, which describes an inflammatory process with increased protein and few lymphocytes, the EEG shows slow waves consistent with an encephalopathic process and MRI unspecified abnormalities in white matter.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The differential diagnosis is broad, and it will be conducted mainly with rapidly progressive dementias<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> and with other causes of encephalopathy. In this regard, the determination of antithyroid antibodies may be useful in neurological conditions when the most common causes have already been ruled out.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a> They are elevated in 100% of cases, with generally normal thyroid function, which assumes an autoimmune basis. However, the pathogenesis is unclear, it is thought that these antibodies are not direct mediators, as its titers do not correlate with the severity of the disease and do not always negativise after response to treatment.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Today, most of the authors consider it an inflammatory central nervous system disease similar to vasculitis.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a> This is supported by the identification of an antigen shared by thyroid and brain, alpha-enolase,<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> concentrated in endothelial cells whose antibodies can be diagnostically useful.30% of patients have other autoimmune diseases, the most described being lupus, rheumatoid arthritis and Sjogren's disease.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a> This would be the first case of Hashimoto's encephalopathy to be involved with primary biliary cirrhosis and vitiligo.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The writer declares no conflicts of interest in the production of this letter.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflict of interest" ] 1 => 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: Peña-Irún Á. Encefalitis de Hashimoto asociada a cirrosis biliar primaria y vitíligo. Med Clin (Barc). 2016;146:e31–e32.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Encefalitis de Hashimoto, a propósito de un caso" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M. Zamora-Elson" 1 => "L. Laborta-Manzón" 2 => "T. Mallor-Bonet" 3 => "V. Villacampa-Claver" 4 => "M. Avellanas-Chavala" 5 => "C. 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