Letter to the Editor
Bickerstaff encephalitis: Case report
Encefalitis de Bickerstaff: presentación de un caso
Raquel Piñar Morales
, Francisco Javier Barrero Hernández
Corresponding author
Servicio de Neurología, Hospital Universitario Clínico San Cecilio, Granada, Spain
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Three days earlier, non-measured fever with increased respiratory secretions were added to the former symptoms. The patient went to the emergency department for general malaise and blurred vision, as well as generalized weakness, predominantly in the left limbs. His condition worsened in the emergency department, developing binocular diplopia, fever up to 38.6 °C and somnolence, requiring orotracheal intubation and admission to the ICU. On examination, the patient showed drowsiness, mild dysarthria, extrinsic ocular motility changes with hypotropia in the right eye, isochoric normoreactive pupils and limitation in the abduction of the left eye with sustained horizontal-torsional nystagmus. He showed mild weakness in the left upper limb with claudication of antigravity activity. Initially, he presented with flexor plantar cutaneous reflex (PCR) and symmetrical non-pathological live deep tendon reflexes (DTR); subsequently, significant dysarthria, generalized enhanced DTR, and bilateral extensor PCR.</p><p id="par0015" class="elsevierStylePara elsevierViewall">A lumbar puncture was performed with normal cytochemistry and a negative microbiological study. The baseline EEG showed irregular base activity, without epileptiform findings. Cranial magnetic resonance imaging (MRI) was normal, with no further findings. The electromyographic/electroneurographic (EMG/ENG) study performed 2 weeks after the onset of symptoms showed no abnormalities. The blink reflex showed an absence of R2 response. Laboratory tests were positive for anti-ganglioside anti-GM1 and anti-GM3 antibodies (Ab). The rest of the study, including serologies, serum total protein and antineuronal antibodies, was negative.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Initially treated with intravenous immunoglobulins, in the absence of a favourable progression, treatment with intravenous corticosteroids was added. After a few days, clinical improvement started, intubation could be withdrawn, and functional recovery started.</p><p id="par0025" class="elsevierStylePara elsevierViewall">BBE is an entity included within the spectrum called anti-GQ1b syndrome, which also includes MFS,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and is believed to be related to Guillain-Barré syndrome (GBS) due to similarities in pathogenesis, clinical features, and ancillary test findings. Its incidence is unknown, although some authors have estimated an annual incidence of 0.078/100,000 inhabitants.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">BBE is a monophasic disease characterized by a subacute onset of ataxia and ophthalmoplegia associated with an alteration in the level of consciousness of variable intensity, pyramidal tract signs (more infrequently hiccups or areflexia) and weakness. Other symptoms that may appear are facial weakness, sensory alterations and dysarthria.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Diagnosis is based on clinical suspicion and the exclusion of other diseases with similar manifestations, such as vertebrobasilar stroke, infectious or autoimmune rhombencephalitis, Wernicke encephalopathy, botulism, myasthenia gravis, a brain stem tumour, pituitary gland stroke, acute disseminated encephalomyelitis, multiple sclerosis, neuro-Behçet's disease, vasculitis, lymphomas, and Creutzfeldt-Jakob disease.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Laboratory findings include albuminocytological dissociation (with elevated cerebrospinal fluid proteins without cellularity), pathological findings on nerve conduction study using EMG/ENG and, less frequently, signal alteration on MRI.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The presence of antiganglioside Ab, specifically IgG anti-GQ1b Ab, is quite common, although positive results have also been described for other antiganglioside Ab,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> including GM1,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> as in the case reported.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The pathogenesis is unknown, but it is thought to be an immune-mediated infection-triggered disease. It shares many similarities with MFS, including positive results for anti-GQ1b Aby, the presence of ophthalmoplegia and/or ataxia. Therefore, it is believed that both may be part of a continuum in which BBE affects the central nervous system.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The relationship with GBS is less clear and it is not known whether they are part of the same clinical spectrum or are distinct entities.</p><p id="par0050" class="elsevierStylePara elsevierViewall">There are no randomized trials regarding treatment; however, both intravenous immunoglobulins and plasmapheresis have been used. The course is usually monophasic, with a good prognosis, and complete remission usually occurs within the first 6 months, although mild symptoms such as dysesthesia, ataxia, or ophthalmoplegia may persist in some patients.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">This entity must be considered when assessing patients with a low level of consciousness, as the infectious history, clinical examination and the determination of antiganglioside Ab can provide the key to establishing an early diagnosis, thus avoiding unnecessary treatment and a favourable prognosis in most patients.</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: Piñar Morales R, Barrero Hernández FJ. Encefalitis de Bickerstaff: presentación de un caso. Med Clin (Barc). 2020. <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.medcli.2020.05.053">https://doi.org/10.1016/j.medcli.2020.05.053</span></p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Bickerstaff’s brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré síndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Odaka" 1 => "N. Yuki" 2 => "M. Yamada" 3 => "M. Koga" 4 => "T. Takemi" 5 => "K. Hirata" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1093/brain/awg233" "Revista" => array:6 [ "tituloSerie" => "Brain." 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