Letter to the Editor
Role of neurophysiological studies in Miller-Fisher syndrome
Papel del estudio neurofisiológico en el síndrome de Miller-Fisher
L. Gabaldón Torres
, C. Badía Picazo, J. Salas Felipe
Corresponding author
Unidad de Neurología y Neurofisiología, Hospital de Dénia, Alicante, Spain
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Gabaldón Torres, C. Badía Picazo, J. Salas Felipe" "autores" => array:3 [ 0 => array:4 [ "nombre" => "L." "apellidos" => "Gabaldón Torres" "email" => array:1 [ 0 => "laura.gabaldon@marinasalud.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "C." "apellidos" => "Badía Picazo" ] 2 => array:2 [ "nombre" => "J." "apellidos" => "Salas Felipe" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Unidad de Neurología y Neurofisiología, Hospital de Dénia, Alicante, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Papel del estudio neurofisiológico en el síndrome de Miller-Fisher" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1377 "Ancho" => 2917 "Tamanyo" => 324750 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Acute active denervation in both orbicularis oculi (more significant on the left side).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Miller-Fisher syndrome (MFS) is a clinical variant of Guillain–Barré syndrome characterised by the triad of ophthalmoparesis, ataxia, and areflexia. Additionally, it is often associated with other symptoms secondary to cranial nerve weakness (facial paralysis, swallowing disorders, etc.), and even with sensory symptoms in the distal limbs.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">It is usually preceded by an infection, most typically a gastrointestinal infection caused by <span class="elsevierStyleItalic">Campylobacter jejuni</span>.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Other triggers have been described, including vaccination, surgical procedures, etc.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Diagnosis tends not to be difficult when patients have the classic triad of symptoms, albumino-cytological dissociation, and neurophysiological findings. Further clues are provided by certain antibodies associated with these dysimmune neuropathies of the peripheral nervous system.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">At times, the presence of other associated symptoms, the absence of Anti-GQ1b, and lack of anomalous results on complementary tests during the acute phase of the syndrome may delay diagnosis and result in requests for unnecessary diagnostic tests.</p><p id="par0025" class="elsevierStylePara elsevierViewall">We present the case of a 35-year-old man with no relevant personal history except for obesity. He experienced an episode of progressive-onset binocular diplopia and gait instability associated with a headache that had lasted 4 to 5 days. The patient reported having gone scuba diving before the onset of symptoms.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The neurological examination performed at the Emergency Department revealed bilateral eyelid ptosis, eyes remaining immobile in a neutral position, reactive bilateral mydriasis, bilateral but predominantly right-sided peripheral facial paralysis, weakness, bilateral elevation of the soft palate with no muscular atrophy or muscle tone alteration, universal areflexia, preserved sensitivity, and ataxic gait. During the hospital stay, he presented high blood pressure and required treatment with intravenous nitrates.</p><p id="par0035" class="elsevierStylePara elsevierViewall">A complete complementary study was performed, including biochemical, immunology (Anti-GQ1b), tumour marker, folate, and cyanocobalamin tests. Results were negative or normal. Serology tests were normal, except for the test for <span class="elsevierStyleItalic">C. jejuni</span> antibodies, which delivered positive results. The CSF study showed albumino-cytological dissociation. Neuroimaging studies (including brain MRI), and intracranial vessel and cerebral blood flow studies all yielded normal results. The neurophysiological study during the acute phase revealed a decrease in M response amplitude in the bilateral facial nerve and abolished blink reflex. An EMG taken 15 days later showed severe demyelinating neuropathy with axonal damage in the facial nerve. While its latency was still high, the previously abolished palpebral reflex (blink reflex) could be observed (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The patient was diagnosed with Miller-Fisher syndrome associated with <span class="elsevierStyleItalic">C. jejuni</span>.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The patient was treated with intravenous immunoglobulins (IGIV) during 5 consecutive days. His facial weakness initially increased to the point of becoming bilateral facial paralysis, but the condition began to subside slowly after a week. Throughout the course of the disease, he presented no swallowing/respiratory disorders or ECG abnormalities, although his diastolic blood pressure was high.</p><p id="par0050" class="elsevierStylePara elsevierViewall">After 3 months, doctors observed clinical improvement and near-complete resolution of facial weakness. Diplopia and ocular motility resolved partially, and the patient experienced no gait abnormalities and no other associated symptoms.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Regarding the predisposing factors, the infection caused by <span class="elsevierStyleItalic">C. jejuni</span> is widely described in the scientific literature. After a long Medline search, however, we did not find any association between this disease and scuba diving, even if the nervous system is frequently affected by decompression problems and one of the divers’ most common symptom is headache.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Although our case does not enable us to consider diving a predisposing factor, we do think that it could facilitate and probably cause other associated symptoms (headache) that were unclear upon initial diagnosis.</p><p id="par0060" class="elsevierStylePara elsevierViewall">While non-reactive bilateral mydriasis is not usually present in MFS, neither is it uncommon. The symptom calls for differential diagnosis, especially in cases of botulism.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Clinical data led us to order complementary studies of the central nervous system in order to rule out processes associated with diving, as well as other processes including cerebral venous sinus thrombosis. Another approach was to examine sociodemographic data, such as canned food consumption, in order to rule out the possibility of botulism, which would have been consistent with the marked cranial nerve involvement and the finding of mydriasis.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Despite the atypical symptoms and the negative result for Anti-GQ1b, findings that are present in more than 90% of MFS patients,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> results from the neurophysiological study during the acute phase helped us establish an early diagnosis and rapidly administer immunomodulatory therapy. In severe cases, this treatment is associated with better clinical progression and, consequently, with a better prognosis.</p><p id="par0075" class="elsevierStylePara elsevierViewall">In general, neurophysiological findings during the acute phase of the disease have not been widely studied, and the subject remains controversial. The study by Calleja et al.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> assessed 5 patients who were diagnosed with MFS. Their results were similar to those from our patient, with a decrease in M response amplitude and abolished palpebral reflex.</p><p id="par0080" class="elsevierStylePara elsevierViewall">In conclusion, we believe neurophysiological studies are necessary during the acute phase of polyneuropathies such as Miller-Fisher syndrome. Results will serve to support the suspected diagnosis from the beginning and unnecessary complementary studies may therefore be avoided.</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: Gabaldón Torres L, Badía Picazo C, Salas Felipe J. Papel del estudio neurofisiológico en el síndrome de Miller-Fisher. Neurología. 2013;28:451–452.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1377 "Ancho" => 2917 "Tamanyo" => 324750 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Acute active denervation in both orbicularis oculi (more significant on the left side).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "An unusual variant of acute idiopathic polyneuritis (syndrome of ophtalmoplegia, ataxia and arreflexia)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "M. 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| 2021 February | 87 | 18 | 105 |
| 2021 January | 68 | 13 | 81 |
| 2020 December | 84 | 17 | 101 |
| 2020 November | 73 | 15 | 88 |
| 2020 October | 75 | 9 | 84 |
| 2020 September | 62 | 13 | 75 |
| 2020 August | 66 | 14 | 80 |
| 2020 July | 68 | 13 | 81 |
| 2020 June | 55 | 6 | 61 |
| 2020 May | 89 | 23 | 112 |
| 2020 April | 44 | 5 | 49 |
| 2020 March | 54 | 6 | 60 |
| 2020 February | 49 | 7 | 56 |
| 2020 January | 59 | 17 | 76 |
| 2019 December | 55 | 7 | 62 |
| 2019 November | 26 | 9 | 35 |
| 2019 October | 43 | 16 | 59 |
| 2019 September | 37 | 4 | 41 |
| 2019 August | 43 | 3 | 46 |
| 2019 July | 52 | 19 | 71 |
| 2019 June | 109 | 33 | 142 |
| 2019 May | 214 | 33 | 247 |
| 2019 April | 77 | 18 | 95 |
| 2019 March | 26 | 6 | 32 |
| 2019 February | 28 | 14 | 42 |
| 2019 January | 21 | 9 | 30 |
| 2018 December | 20 | 8 | 28 |
| 2018 November | 21 | 3 | 24 |
| 2018 October | 30 | 4 | 34 |
| 2018 September | 29 | 6 | 35 |
| 2018 August | 16 | 6 | 22 |
| 2018 July | 14 | 0 | 14 |
| 2018 June | 14 | 2 | 16 |
| 2018 May | 10 | 10 | 20 |
| 2018 April | 8 | 1 | 9 |
| 2018 March | 28 | 1 | 29 |
| 2018 February | 21 | 2 | 23 |
| 2018 January | 10 | 4 | 14 |
| 2017 December | 46 | 4 | 50 |
| 2017 November | 9 | 4 | 13 |
| 2017 October | 18 | 4 | 22 |
| 2017 September | 13 | 8 | 21 |
| 2017 August | 18 | 9 | 27 |
| 2017 July | 13 | 8 | 21 |
| 2017 June | 14 | 17 | 31 |
| 2017 May | 14 | 22 | 36 |
| 2017 April | 18 | 4 | 22 |
| 2017 March | 10 | 29 | 39 |
| 2017 February | 14 | 4 | 18 |
| 2017 January | 14 | 6 | 20 |
| 2016 December | 12 | 12 | 24 |
| 2016 November | 17 | 8 | 25 |
| 2016 October | 16 | 4 | 20 |
| 2016 September | 12 | 6 | 18 |
| 2016 August | 14 | 6 | 20 |
| 2016 July | 8 | 1 | 9 |
| 2016 June | 18 | 14 | 32 |
| 2016 May | 16 | 17 | 33 |
| 2016 April | 22 | 6 | 28 |
| 2016 March | 22 | 15 | 37 |
| 2016 February | 12 | 13 | 25 |
| 2016 January | 10 | 2 | 12 |
| 2015 December | 8 | 6 | 14 |
| 2015 November | 13 | 4 | 17 |
| 2015 October | 22 | 7 | 29 |
| 2015 September | 21 | 7 | 28 |
| 2015 August | 5 | 4 | 9 |
| 2015 July | 5 | 7 | 12 |
| 2015 June | 8 | 1 | 9 |
| 2015 May | 6 | 3 | 9 |
| 2015 April | 9 | 14 | 23 |
| 2015 March | 13 | 4 | 17 |
| 2015 February | 14 | 3 | 17 |
| 2015 January | 28 | 9 | 37 |
| 2014 December | 34 | 9 | 43 |
| 2014 November | 21 | 4 | 25 |
| 2014 October | 21 | 2 | 23 |
| 2014 September | 30 | 2 | 32 |
| 2014 August | 23 | 9 | 32 |
| 2014 July | 35 | 8 | 43 |
| 2014 June | 16 | 5 | 21 |
| 2014 May | 15 | 5 | 20 |
| 2014 April | 16 | 2 | 18 |
| 2014 March | 26 | 4 | 30 |
| 2014 February | 12 | 2 | 14 |
| 2014 January | 17 | 3 | 20 |
| 2013 December | 18 | 3 | 21 |
| 2013 November | 41 | 13 | 54 |
Show all
