Letter to the Editor
Miller-Fisher syndrome associated with acute motor axonal neuropathy: Clinic-immunological correlation
Síndrome de Miller-Fisher asociado a neuropatía axonal motora aguda: correlación clínico-inmunológica
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Marked, unilateral, low uptake of the right striatum.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "F. Pérez Errazquin, M.J. Gomez Heredia" "autores" => array:2 [ 0 => array:2 [ "nombre" => "F." "apellidos" => "Pérez Errazquin" ] 1 => array:2 [ "nombre" => "M.J." 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Genetic mutations described both in the work of Saporta et al<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> and in Spanish patients are shown in bold (see text for details, especially in relation to the priorities of molecular study).</p> <p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">AD: autosomal dominant; AR: autosomal recessive; ♂→♂: male–male transmission; ♂→<span class="elsevierStyleSup">|</span>♂: no evidence of male–male transmission.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "J. Berciano, T. Sevilla, C. Casasnovas, R. Sivera, J.J. Vílchez, J. Infante, C. Ramón, A.L. Pelayo-Negro, I. Illa" "autores" => array:10 [ 0 => array:2 [ "nombre" => "J." "apellidos" => "Berciano" ] 1 => array:2 [ "nombre" => "T." "apellidos" => "Sevilla" ] 2 => array:2 [ "nombre" => "C." "apellidos" => "Casasnovas" ] 3 => array:2 [ "nombre" => "R." "apellidos" => "Sivera" ] 4 => array:2 [ "nombre" => "J.J." 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Madrid Rodríguez, J. Martínez Antón, M. Núñez Castaín, J.M. Ramos Fernández" "autores" => array:4 [ 0 => array:3 [ "nombre" => "A." "apellidos" => "Madrid Rodríguez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:3 [ "nombre" => "J." "apellidos" => "Martínez Antón" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "M." "apellidos" => "Núñez Castaín" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:4 [ "nombre" => "J.M." "apellidos" => "Ramos Fernández" "email" => array:1 [ 0 => "jmramos@doctor.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Sección de Neuropediatría, Servicio de Pediatría, Hospital Regional Universitario Carlos Haya, Hospital Materno-Infantil, Málaga, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neurofisiología, Hospital Regional Universitario Carlos Haya, Hospital Materno-Infantil, Málaga, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Miller-Fisher asociado a neuropatía axonal motora aguda: correlación clínico-inmunológica" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Miller-Fisher syndrome (MFS) is an extremely rare, autoimmune, axonal polyradiculoneuropathy which takes place during childhood. It is characterised by the clinical triad of ataxia, ophthalmoplegia and arreflexia.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The annual incidence of Miller-Fisher syndrome is 0.09 cases per 100<span class="elsevierStyleHsp" style=""></span>000 inhabitants. The few published series that exist with children account for approximately 9% of the total of acute polyradiculoneuropathy cases.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> As in the case of Guillain–Barré syndrome, it is often triggered by certain strains of <span class="elsevierStyleItalic">Campylobacter jejuni</span> that induce the formation of anti-GQ1b antiganglioside, although there have also been cases described involving <span class="elsevierStyleItalic">Haemophilus influenzae</span> and <span class="elsevierStyleItalic">Mycoplasma pneumoniae</span>. Anti-GQ1b antibodies are elevated in 90–97% of Miller-Fisher syndrome cases. These antibodies recognise epitopes that are expressed specifically in the nodal regions of the oculomotor nerves, in the dorsal root ganglia and in the cerebellar neurons. All these structures are responsible for the symptoms of Miller-Fisher syndrome.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Occasionally, Miller-Fisher syndrome and Guillain-Barré syndrome in its demyelinating, acute, motor axonal and acute sensorimotor variants may have an overlapping clinical spectrum, depending on the immunopathological cause. Moreover, motor axonal forms generally respect cranial nerves and present a predominantly distal involvement. Very few cases of this type of presentation have been studied in the light of current knowledge, improved antibody detection techniques and purification of new antigens from the nervous system. We present a study of a patient suffering from Miller-Fisher variant associated with an acute peripheral case of acute motor axonal neuropathy (AMAN). We analysed the antiganglioside antibody pattern and its correlation with the symptoms, as well as the evolution and response to treatment with intravenous immunoglobulin.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a boy aged 4 years and 9 months, who was admitted to our hospital due to generalised weakness with inability for ambulation, gait instability and palpebral oedema with right ptosis. These symptoms had a progressive clinical evolution of 2 weeks. The patient had suffered an episode of gastroenteritis of unknown aetiology 15 days earlier.</p><p id="par0020" class="elsevierStylePara elsevierViewall">On examination at admission the patient was prostrate, with sensation of acute illness, highlighting a mild bilateral palpebral oedema with right ptosis. At the neurological level, he was conscious, alert, and responsive, with a clear sensory spectrum. He also presented ophthalmoplegia affecting the third, fourth and sixth cranial nerves, inability for vertical and horizontal visual tracking and bilateral facial paresis. In addition, he suffered generalised hypotonia with proximal predominance, inability to sit and walk, decreased strength which was especially marked in the lower limbs, and a slightly asymmetrical balance of the right leg over the left, 1/5 and 2/5, respectively. He also presented universal arreflexia, with seemingly preserved thermo-algesic and proprioceptive sensitivity. No cerebellar signs, tremor, dysmetria or dysdiadochokinesia were identified. There were no meningeal signs.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Complementary tests highlighted albumino-cytological dissociation in the cerebrospinal fluid with protein levels of 0.8<span class="elsevierStyleHsp" style=""></span>g/l and 5 mononuclear cells per mm<span class="elsevierStyleSup">3</span>. Neuroimaging tests (cervical and thoracolumbar MRI scans) showed no morphological changes or signal alterations in the brain, brainstem, spinal cord or cauda equina in T-1, T-2 or FLAIR weighted sequences.</p><p id="par0030" class="elsevierStylePara elsevierViewall">In the initial neurophysiological study, the electromyograms of the upper limb (deltoid, biceps and extensor digitorum muscles) and lower limb (rectus femoris, anterior tibialis and gastrocnemius muscles) revealed a neurogenic pattern. There was spontaneous denervation activity with fibrillations and positive waves, highly deficient evoked motor conduction pathways that were more pronounced in the proximal muscles of the upper limb being examined, and motor unit potentials of long duration and great amplitude, with an increased proportion of polyphasic motor unit potentials. The electroneurogram presented impaired motor conduction with reduced amplitude. Distal latency and conduction velocity remained at normal parameters at the level of both facial nerves, right median nerve, right ulnar nerve and both peroneal nerves. Sensory conduction was within normal limits in both speed and amplitude and was, therefore, compatible with motor axonal polyradiculopathy.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The seroimmunological study of antiglycolipid antibodies conducted by enzyme immunoassay (ELISA) detected the presence of IgM antibodies against ganglioside GQ1b at a titre of 1/1500 and IgG positivity against ganglioside GM1 at a titre of 1/500 and IgM against antigen GM2 at a titre of 1/3000. Determination of the remaining antiglycolipid antibodies against GM3, asialo GM1, GD1a, GD1b, GD3, GT1b, sulphatide and globoside were negative.</p><p id="par0040" class="elsevierStylePara elsevierViewall">We performed stool culture for <span class="elsevierStyleItalic">Campylobacter jejuni</span>, which resulted negative. CRP in blood for herpes group viruses was negative. The patient was diagnosed with Miller-Fisher syndrome and associated acute motor axonal neuropathy.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The patient was treated with intravenous immunoglobulin at doses of 2<span class="elsevierStyleHsp" style=""></span>g/kg (400<span class="elsevierStyleHsp" style=""></span>mg/kg/day for 5 days) and early motor rehabilitation, with good clinical and neurophysiological response. One month after starting treatment, the clinical examination revealed partial recovery of oculomotor and facial reflexes, unaided sitting, aided standing and paraparetic walking with some aid. In the control neurophysiological study conducted 3 months later, the electromyograms of the orbicularis oculi and the right deltoid revealed an absence of spontaneous activity. Furthermore, voluntary movements at maximum effort were slightly deficient in the orbicularis oculi and without significant deficit in the deltoid. Motor unit potentials were normal. The control electroneurogram showed motor conduction involvement of the right and left facial nerves, with decreased amplitude in both evoked potentials, but predominantly on the left. The only finding in connection with the initial neurophysiological examination was peripheral neuropathy of the facial nerves, predominantly on the left side, possibly at the level of the intrapetrous pathway. However, this had a lesser degree than in the initial examination and the remainder of the neurophysiological study was normal.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Current knowledge of acute polyradiculoneuropathies indicates that these entities are acquired as the result of an aberrant immune response secondary to a triggering event. This event could be infection, vaccination, malignancy or some other autoimmune stimulus. The variety of antibodies formed determines the subsequent pathological outcome.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Thus, the presence of anti-GQ1b leads to the involvement of oculomotor nerves since antigen GQb1 is specifically expressed in the nodal regions of the oculomotor nerves, the dorsal root ganglia and the cerebellar neurons. Furthermore, acute neuropathy characterised by cervical–pharyngeal–brachial paralysis or bulbar dysfunction has been recognised as a variant of Guillain–Barré syndrome. In addition, a recent clinical study has shown that cervical–pharyngeal–brachial paralysis, Miller-Fisher syndrome and Bickerstaff encephalitis form a continuous clinical spectrum.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> A specific anti-GT1a antibody without GQ1b reactivity is essential for the development of bulbar paralysis in patients with Guillain-Barré syndrome. The glossopharyngeal nerve and vagus nerve contain GQ1b and GT1a,<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> but the presence of GT1a has not been demonstrated in human peripheral nerves. It is likely that specific anti-GD1b antibodies cause ataxia in Guillain–Barré syndrome.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Antigens GM1, GM1b, GD1a, and GalNAc-GD1a are also typical of peripheral motor nerve axolemma at the level of the Ranvier nodes (in animal models). Consequently, the development of their respective antibodies leads to AMAN.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Antibodies against GM2 have been described in cases of cranial nerve paralysis, as well as in cases of sensory involvement associated with herpes group virus infections.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> A frequent association between the presence of anti-GM2 and facial paralysis has also been reported.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The clinical characteristics of our patient (oculomotor cranial nerve involvement, facial involvement and motor axonal neuropathy without demyelination) had a consistent correlation with his immunophenotype, namely anti-GQb1 with ophthalmoplegia, anti-GM1 with motor axonal involvement and anti-GM2 with facial paresis. However, previous publications have reported greater variability in the behaviour of the latter antibody, since it has also been associated with demyelinating and high cranial nerve symptoms, always with facial involvement. This antigen is located both in the axon and in the myelin around Ranvier nodes.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Antiganglioside antibodies play an important role in the pathophysiology of AMAN-type polyneuropathy and Miller-Fisher syndrome. Antiganglioside antibodies cause nerve damage through complement activation or function impairment of voltage-dependent calcium and sodium channels. The grouped (clustered) epitopes from the complexes of 2 gangliosides in the cell membrane can be recognised by serum antibodies in Guillain-Barré syndrome and Miller-Fisher syndrome and may regulate the accessibility and avidity of antiganglioside antibodies. The glycolipid configuration or specific distribution of ganglioside receptors in the peripheral nervous system may also influence the pathogenic effect in Guillain–Barré syndrome and Miller-Fisher syndrome.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Theoretically, involvement of the type of immunoglobulin elicited by the previous autoimmune stimulus is connected with the development of IgM in initial stages. In addition, the possibility exists of triggering “membrane attack complex” via complement activation. This would lead to more severe and difficult to control damage than that caused by IgG affecting sodium and calcium channels. It is possible and reasonable that treatment with immunoglobulins in early stages would improve prognosis and long-term evolution by preventing progression of autoimmune activation, as in the case of our patient.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Madrid Rodríguez A, et al. Síndrome de Miller-Fisher asociado a neuropatía axonal motora aguda: correlación clínico-inmunológica. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 3 | 1 | 4 |
| 2024 October | 39 | 10 | 49 |
| 2024 September | 41 | 8 | 49 |
| 2024 August | 58 | 3 | 61 |
| 2024 July | 54 | 8 | 62 |
| 2024 June | 35 | 5 | 40 |
| 2024 May | 50 | 8 | 58 |
| 2024 April | 42 | 10 | 52 |
| 2024 March | 35 | 17 | 52 |
| 2024 February | 35 | 10 | 45 |
| 2024 January | 48 | 9 | 57 |
| 2023 December | 33 | 14 | 47 |
| 2023 November | 43 | 13 | 56 |
| 2023 October | 52 | 20 | 72 |
| 2023 September | 27 | 11 | 38 |
| 2023 August | 17 | 3 | 20 |
| 2023 July | 17 | 8 | 25 |
| 2023 June | 14 | 3 | 17 |
| 2023 May | 15 | 5 | 20 |
| 2023 April | 28 | 6 | 34 |
| 2023 March | 19 | 7 | 26 |
| 2023 February | 24 | 2 | 26 |
| 2023 January | 20 | 8 | 28 |
| 2022 December | 15 | 9 | 24 |
| 2022 November | 27 | 17 | 44 |
| 2022 October | 18 | 12 | 30 |
| 2022 September | 15 | 15 | 30 |
| 2022 August | 30 | 14 | 44 |
| 2022 July | 17 | 9 | 26 |
| 2022 June | 11 | 8 | 19 |
| 2022 May | 21 | 14 | 35 |
| 2022 April | 39 | 11 | 50 |
| 2022 March | 38 | 13 | 51 |
| 2022 February | 54 | 7 | 61 |
| 2022 January | 48 | 13 | 61 |
| 2021 December | 62 | 16 | 78 |
| 2021 November | 60 | 10 | 70 |
| 2021 October | 48 | 15 | 63 |
| 2021 September | 25 | 18 | 43 |
| 2021 August | 32 | 8 | 40 |
| 2021 July | 28 | 8 | 36 |
| 2021 June | 24 | 10 | 34 |
| 2021 May | 38 | 6 | 44 |
| 2021 April | 121 | 15 | 136 |
| 2021 March | 115 | 15 | 130 |
| 2021 February | 72 | 8 | 80 |
| 2021 January | 72 | 12 | 84 |
| 2020 December | 47 | 9 | 56 |
| 2020 November | 51 | 6 | 57 |
| 2020 October | 42 | 11 | 53 |
| 2020 September | 31 | 14 | 45 |
| 2020 August | 47 | 11 | 58 |
| 2020 July | 44 | 13 | 57 |
| 2020 June | 33 | 14 | 47 |
| 2020 May | 39 | 16 | 55 |
| 2020 April | 53 | 9 | 62 |
| 2020 March | 41 | 10 | 51 |
| 2020 February | 52 | 9 | 61 |
| 2020 January | 48 | 16 | 64 |
| 2019 December | 42 | 10 | 52 |
| 2019 November | 51 | 8 | 59 |
| 2019 October | 37 | 6 | 43 |
| 2019 September | 50 | 5 | 55 |
| 2019 August | 45 | 12 | 57 |
| 2019 July | 19 | 9 | 28 |
| 2019 June | 69 | 12 | 81 |
| 2019 May | 106 | 17 | 123 |
| 2019 April | 53 | 27 | 80 |
| 2019 March | 21 | 10 | 31 |
| 2019 February | 26 | 10 | 36 |
| 2019 January | 29 | 9 | 38 |
| 2018 December | 38 | 1 | 39 |
| 2018 November | 28 | 4 | 32 |
| 2018 October | 25 | 9 | 34 |
| 2018 September | 40 | 3 | 43 |
| 2018 August | 35 | 5 | 40 |
| 2018 July | 12 | 4 | 16 |
| 2018 June | 20 | 1 | 21 |
| 2018 May | 17 | 6 | 23 |
| 2018 April | 23 | 1 | 24 |
| 2018 March | 10 | 5 | 15 |
| 2018 February | 3 | 2 | 5 |
| 2018 January | 17 | 3 | 20 |
| 2017 December | 15 | 0 | 15 |
| 2017 November | 17 | 3 | 20 |
| 2017 October | 8 | 4 | 12 |
| 2017 September | 13 | 6 | 19 |
| 2017 August | 14 | 6 | 20 |
| 2017 July | 16 | 3 | 19 |
| 2017 June | 39 | 9 | 48 |
| 2017 May | 27 | 8 | 35 |
| 2017 April | 22 | 12 | 34 |
| 2017 March | 25 | 23 | 48 |
| 2017 February | 22 | 1 | 23 |
| 2017 January | 15 | 7 | 22 |
| 2016 December | 22 | 6 | 28 |
| 2016 November | 28 | 10 | 38 |
| 2016 October | 39 | 7 | 46 |
| 2016 September | 32 | 8 | 40 |
| 2016 August | 44 | 9 | 53 |
| 2016 July | 48 | 2 | 50 |
| 2016 June | 33 | 12 | 45 |
| 2016 May | 35 | 21 | 56 |
| 2016 April | 41 | 15 | 56 |
| 2016 March | 60 | 30 | 90 |
| 2016 February | 43 | 24 | 67 |
| 2016 January | 33 | 21 | 54 |
| 2015 December | 26 | 14 | 40 |
| 2015 November | 17 | 10 | 27 |
| 2015 October | 33 | 15 | 48 |
| 2015 September | 23 | 10 | 33 |
| 2015 August | 18 | 11 | 29 |
| 2015 July | 20 | 4 | 24 |
| 2015 June | 14 | 9 | 23 |
| 2015 May | 13 | 5 | 18 |
| 2015 April | 10 | 11 | 21 |
| 2015 March | 25 | 10 | 35 |
| 2015 February | 21 | 10 | 31 |
| 2015 January | 35 | 11 | 46 |
| 2014 December | 48 | 9 | 57 |
| 2014 November | 28 | 6 | 34 |
| 2014 October | 46 | 11 | 57 |
| 2014 September | 35 | 3 | 38 |
| 2014 August | 48 | 8 | 56 |
| 2014 July | 54 | 6 | 60 |
| 2014 June | 41 | 5 | 46 |
| 2014 May | 23 | 0 | 23 |
| 2014 April | 23 | 6 | 29 |
| 2014 March | 26 | 4 | 30 |
| 2014 February | 21 | 5 | 26 |
| 2014 January | 20 | 2 | 22 |
| 2013 December | 22 | 5 | 27 |
| 2013 November | 24 | 7 | 31 |
| 2013 October | 44 | 6 | 50 |
| 2013 September | 50 | 7 | 57 |
| 2013 August | 44 | 5 | 49 |
| 2013 July | 25 | 7 | 32 |
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