Guillain-Barré syndrome with overlap between the finger drop variant and acute bulbar palsy: a case report

Guillain-Barré syndrome with overlap between the finger drop variant and acute bulbar palsy: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Guillain-Barré syndrome with overlap between the finger drop variant and acute bulbar palsy: a case report Shota Ito, Satoshi Yokoi, Yuki Fukami, Ayumi Uchibori, Masahisa Katsuno This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4211221/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 23 Oct, 2024 Read the published version in BMC Neurology → Version 1 posted 14 You are reading this latest preprint version Abstract Background Guillain-Barré syndrome (GBS) encompasses diverse variants, and the overlap between these variants often makes the diagnosis of GBS difficult. We report a case of GBS overlapping with both the finger drop variant and acute bulbar palsy. Case presentation: An 81-year-old man presented with dysarthria, dysphagia, and upper limb weakness. The patient exhibited impaired tongue protrusion and the finger drop sign, whereas other cranial nerve functions, leg strength, touch sensation, and proprioception were normal. Brachioradialis and triceps reflexes were diminished. Cerebrospinal fluid analysis revealed elevated protein levels with normal cell counts. Nerve conduction studies showed reduced amplitudes and decreased velocities in the median and ulnar nerves. As GBS was suspected, we initiated intravenous immunoglobulin treatment, resulting in gradual improvement within 3 weeks. Serum IgG antibodies to gangliosides tested positive for GM1, asialo-GM1, GT1a, GD1b, and GQ1b. On the basis of the monophasic disease course and anti-ganglioside antibodies, we confirmed the diagnosis of GBS. Conclusion This case is the first reported instance of an overlap between the finger drop variant and acute bulbar palsy in GBS, highlighting the importance of considering GBS when patients present with a combination of atypical symptoms. Anti-ganglioside antibodies may assist in distinguishing these complex symptoms. Guillain-Barré syndrome finger drop sign bulbar palsy anti-ganglioside antibody Figures Figure 1 Figure 2 Figure 3 Background Guillain-Barré syndrome (GBS) is an acute autoimmune disorder that predominantly affects the peripheral nervous system and is characterized by a rapidly progressing course. Classical GBS typically begins with weakness in the legs that progressively ascends to involve the upper limbs, and in more severe cases, the respiratory muscles and cranial nerves ( 1 ). GBS is characterized by a variety of subtypes, each defined by specific clinical features, a wide variety of which have been highlighted over time. For instance, the ‘finger drop sign’ was recently identified as a variant of GBS, known as the finger drop variant (FDv) ( 2 ). It is defined by prominent distal weakness in the upper limbs, predominantly affecting the fingers or wrist extensors more than the flexors. This variant usually exhibits no weakness or only mild weakness in the lower limbs. Because of its focal weakness and low disease frequency, this variant poses diagnostic challenges. Another important clinical symptom in patients with GBS is acute bulbar palsy. This symptom is typical of the pharyngeal-cervical-brachial (PCB) variant, which is typified by oropharyngeal and cervicobrachial weakness, without lower limb involvement ( 3 ). However, some patients with GBS primarily present with acute bulbar palsy without significant neck and brachial weakness, indicating another GBS variant ( 3 ). Such variable manifestation often complicates the diagnostic process, especially in the early stage. Furthermore, overlap between these variants sometimes occurs in patients with GBS, and this can make the diagnosis extremely complicated for clinicians. However, anti-ganglioside antibodies may help elucidate the clinical subtypes ( 1 ). It was reported that IgG anti-GM1 antibodies, as well as the ganglioside complex of anti-GM1 antibodies with phosphatidic acid, are linked to typical GBS and FDv ( 2 ). Similarly, anti-GD1b antibodies are commonly associated with sensory ataxic neuropathy, whereas anti-GT1a antibodies are related to PCB variants and acute bulbar palsy in patients with GBS ( 1 , 4 ). Furthermore, anti-GQ1b antibodies are often associated with Miller Fisher syndrome (MFS) ( 1 ). While definitive associations between these variants and specific anti-ganglioside antibodies have not been firmly established, the presence of these antibodies is often informative for the diagnosis of GBS. In this report, we present a case of a patient with GBS who exhibited a combination of quite atypical features, specifically, the finger drop sign and acute bulbar palsy due to tongue weakness. A precise neurological examination was important for the accurate diagnosis of this case in the early stage. Anti-ganglioside antibodies were useful to explain the phenotypes. Case Presentation An 81-year-old man with a history of appendix cancer that recurred following treatment with cetuximab and encorafenib was admitted to a neurology department because of dysarthria, dysphagia, and weakness of the upper limbs lasting for 3 days. The patient had received no recent vaccinations, and had no prior infection, diarrhea, or upper respiratory symptoms. Neurological examination of the cranial nerves revealed dysarthria and dysphagia with an inability to protrude the tongue beyond the dental arch. There were no abnormalities in ocular movements and facial strength. The gait was normal, and no ataxia was observed; additionally, touch sensation and proprioception were also normal. Manual muscle testing conducted using the Medical Research Council scale revealed muscle weakness in the distal upper limbs. Bilateral wrist extensors and flexors scored two on the right and three on the left. Finger extensors were notably weak, with a uniform rating of two bilaterally, and the finger drop sign being shown (Fig. 1 a). By contrast, the finger flexors exhibited only mild weakness, rated at three on the right and four on the left. The iliopsoas and hamstring muscles showed mild weakness, each scoring four. Muscles in other areas of the lower limbs were found to be at full strength. Tendon reflexes were diminished in the brachioradialis and triceps, but were normal in other sites. The blood test results were normal with no anti-SSA antibody, anti-SSB antibody, myeloperoxidase and proteinase 3 anti-neutrophil cytoplasmic antibodies, anti-acetylcholine receptor antibody, or anti-muscle-specific tyrosine kinase antibody. Nerve conduction studies revealed low amplitudes and deteriorated velocities with proximal stimulation in the median and ulnar motor nerves, with the results otherwise being within normal limits (Table 1 and Fig. 2 ). Respiratory function tests demonstrated a vital capacity of 71%. A cerebral spinal fluid test showed elevated protein levels of 48 mg/dL and normal cell counts, with five cells per mm³. Brain MRI and cervical contrast-enhanced MRI were normal. Tongue pressure quantified with a balloon-type tongue pressure measurement device (TPM-02; JMS Co. Ltd, Hiroshima, Japan) was low, at an average of 10.44 kPa over ten trials, with this being only 33% of the average of 32 kPa for a 70-year-old population ( 5 ). Videoendoscopic evaluation of swallowing revealed reduced pharyngeal contractility and normal laryngeal function. Table 1 The results of nerve conduction studies on day 1 and 21 after admission day 1 day 21 Nerve Stimulation Latency(ms) CMAP amp(mV) Velocity(m/s) Latency(ms) CMAP amp(mV) Velocity(m/s) Rt. median Wrist 3.1 6.7 3.1 6.1 Elbow 7.9 2.2 47.0 8.1 3.4 47.0 Rt. ulnar Wrist 2.7 7.7 2.5 7.1 Below elbow 7.0 4.8 53.0 7.2 6.1 48.0 Above elbow 10.3 4.6 42.0 9.7 6.0 47.0 Rt. peroneal Ankle 6.1 1.9 5.8 3.1 Fibular head 15.2 1.1 40.0 14.4 1.8 39.0 Rt. tibial Ankle 4.5 8.3 3.8 9.8 Pop fossa 14.4 4.0 41.0 14.4 5.0 41.0 On the first day, the study revealed low amplitude and reduced conduction velocities in the proximal segments of the median and ulnar motor nerves. By the 21st day, there was a slight improvement in the amplitude of these nerves. As a result, we strongly suspected GBS because of the acute onset of the mostly symmetric pattern of limb and cranial nerve weakness with areflexia and albuminocytologic dissociation. Consequently, intravenous immunoglobulin therapy (0.4 g/day for 5 consecutive days), rehabilitation, and fluid administration were initiated on the day following admission. After the treatment, the patient’s symptoms gradually improved. By the 17th day of admission, there was a gradual improvement in the finger drop sign (Fig. 1 b), and the patient was able to resume normal oral intake. The improvement in finger extension was slower than that of flexion, in line with the severity at onset. The patient’s tongue pressure had improved to 23.56 kPa, 74% relative to the normal value for a 70-year-old population (Fig. 3 ). On the 21st day of admission, we again conducted nerve conduction studies (Table 1 and Fig. 2 ), which showed a slight recovery in amplitude and a mild decrease in velocities, in line with acute motor axonal neuropathy (AMAN) ( 6 – 8 ). An enzyme-linked immunosorbent assay (ELISA) of serum IgG antibodies to gangliosides revealed positive results for GM1, asialo-GM1, GD1b, GT1a, and GQ1b, with titers of 0.422, 0.230, 0.404, and 0.452, respectively. On the 31st day after admission, the patient was discharged from the hospital. Given the monophasic disease course and the results of the nerve conduction studies, a diagnosis of AMAN, a subtype of GBS, was established. Discussion and Conclusion The patient presented with the finger drop sign and acute bulbar palsy, which are both relatively rare manifestations of GBS. First, the finger drop sign is a distinctive feature observed in this case. Recent research defined the FDv as corresponding to the AMAN type in nerve conduction studies ( 2 ). Initially, our patient’s nerve conduction studies demonstrated low amplitude, suggesting a conduction block. However, early reversible conduction failure is a known phenomenon in GBS ( 9 ), with this abnormality often being observed in the early stage of AMAN type. In subsequent nerve conduction studies, the conduction velocities returned to normal, confirming the AMAN classification of our case according to the Ho criteria, Hadden criteria, and Uncini criteria ( 6 – 8 ). While our case fulfills the definition of FDv, including positive anti-GM1 antibodies and AMAN type, it is important to note that the presence of a combination of positive anti-GT1a antibodies in FDv has not been previously reported. The occurrence of acute bulbar palsy in GBS is frequently associated with anti-GT1a antibodies ( 10 ). Interestingly, there are no specific studies that have conclusively identified which cranial nerves are predominantly involved in acute bulbar palsy in patients with GBS with positive anti-GT1a antibodies ( 10 , 11 ). Our patient exhibited pronounced tongue weakness at the onset of GBS, which improved rapidly following IVIg treatment. The improvement in tongue pressure was associated with amelioration of the dysarthria and dysphasia, suggesting that tongue pressure may reflect the overall severity of GBS. Tongue weakness is reported to be correlated with respiratory function in GBS ( 12 ). As GT1a is reported to be expressed in the glossopharyngeal nerve, vagal nerve, human cerebrum, and hypoglossal nerve, the presence of anti-GT1a antibodies raises the possibility that the effect of these antibodies on the hypoglossal nerve could cause the unique tongue weakness ( 4 , 13 ). Measuring tongue strength can be a valuable tool to not only assess the severity and progression of bulbar palsy, but to also determine the targets of intervention in rehabilitation. In our case, anti-GD1b and anti-GQ1b antibodies were also positive. While anti-GD1b antibodies indicate sensory ataxic neuropathy and anti-GQ1b antibodies are typically indicative of MFS, the clinical presentation of our patient did not exhibit ataxia and did not align with MFS ( 1 , 14 ). Despite the occurrence of anti-GD1b and GQ1b antibodies in MFS and sensory ataxic neuropathy cases, it is important to consider the possibility of cross reactivity ( 4 , 15 , 16 ). The absence of typical MFS symptoms and reduced deep sensation indicated that the presence of anti-GD1b and anti-GQ1b antibodies in this case may have been due to cross reactivity to anti-GM1 and anti-GT1a antibodies, respectively. In addition, while the patient had appendix cancer, there was low probability of paraneoplastic syndrome because of his monophasic disease course and marked response to IVIg treatments. Our case exhibited a rare combination of clinical features, namely, acute bulbar palsy and the finger drop sign. The simultaneous presence of these symptoms, coupled with the detection of both anti-GT1a and anti-GM1 antibodies, highlights the intricacies and diagnostic challenges associated with overlapping subtypes of GBS, underscoring the need for clinicians to consider the possibility of such overlapping syndromes. Our case particularly suggests an association between anti-GT1a antibody and its impact on the hypoglossal nerve. This finding suggests that measuring tongue strength could be a valuable method for assessing clinical progression and determining appropriate rehabilitation strategies. To disentangle the complexities of these overlapping subtypes, further research is essential to elucidate the distinct clinical subtypes of GBS, their specific associations with anti-ganglioside antibodies, and the mechanisms underlying these associations. Abbreviations GBS: Guillain-Barré syndrome FDv: finger drop variant PCB: Pharyngeal-cervical-brachial MFS: Miller Fisher syndrome MRI: Magnetic resonance imaging IVIg: Intravenous immunoglobulin ELISA: Enzyme-linked immunosorbent assay AMAN: Acute motor axonal neuropathy Declarations Acknowledgments: Not applicable. Funding: None. Availability of data and materials: All data analyzed are included in the published article. Author’s contributions: Shota Ito, Satoshi Yokoi, and Yuki Fukami drafted the manuscript. Ayumi Uchibori measured serum IgG antibodies against gangliosides. Masahisa Katsuno reviewed and revised the manuscript. All authors read and approved the final manuscript. Ethics approval and consent to participate: Written informed consent was obtained from the patient. Consent for publication: The authors have obtained written informed consent from the patient for the publication of this paper. Competing interests: None. References Wakerley BR, Uncini A, Yuki N, GBS Classification Group, GBS Classification Group. Guillain-Barré and Miller Fisher syndromes--new diagnostic classification. Nat Rev Neurol. 2014 Sep;10(9):537–44. Yoon B-A, Ha D-H, Park HT, Kusunoki S, Kuwahara M, Lee JH, et al. Finger drop sign as a new variant of acute motor axonal neuropathy. Muscle Nerve. 2021 Mar;63(3):336–43. Wakerley BR, Yuki N. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry. 2014 Mar;85(3):339–44. Koga M, Yoshino H, Morimatsu M, Yuki N. Anti-GT1a IgG in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 2002 Jun;72(6):767–71. Utanohara Y, Hayashi R, Yoshikawa M, Yoshida M, Tsuga K, Akagawa Y. Standard values of maximum tongue pressure taken using newly developed disposable tongue pressure measurement device. Dysphagia. 2008 Sep;23(3):286–90. Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, et al. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995 Jun 1;118 ( Pt 3)(3):597–605. Hadden RD, Cornblath DR, Hughes RA, Zielasek J, Hartung HP, Toyka KV, et al. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Ann Neurol. 1998 Nov;44(5):780–8. Uncini A, Kuwabara S. Electrodiagnostic criteria for Guillain-Barrè syndrome: a critical revision and the need for an update. Clin Neurophysiol. 2012 Aug;123(8):1487–95. Chan Y-C, Punzalan-Sotelo AM, Kannan TA, Shahrizaila N, Umapathi T, Goh EJH, et al. Electrodiagnosis of reversible conduction failure in Guillain-Barré syndrome. Muscle Nerve. 2017 Nov;56(5):919–24. Kim JK, Kim B-J, Shin HY, Shin KJ, Nam T-S, Oh J, et al. Acute bulbar palsy as a variant of Guillain-Barré syndrome. Neurology. 2016 Feb 23;86(8):742–7. Garg N, Yuki N, Park SB, Barnett MH, Kiernan MC. Acute bulbar, neck and limb weakness with monospecific anti‐GT1a antibody: A rare localized subtype of Guillain‐Barré sydnrome. Muscle Nerve. 2016 Jan;53(1):143–6. Orlikowski D, Terzi N, Blumen M, Sharshar T, Raphael JC, Annane D, et al. Tongue weakness is associated with respiratory failure in patients with severe Guillain-Barré syndrome. Acta Neurol Scand. 2009 Jun;119(6):364–70. Yoshino H, Harukawa H, Asano A. IgG antiganglioside antibodies in Guillain–Barre syndrome with bulbar palsy. J Neuroimmunol. 2000, Jun 26;105(2):195–201. Susuki K, Yuki N, Hirata K. Fine specificity of anti-GQ1b IgG and clinical features. J Neurol Sci. 2001 Mar 15;185(1):5–9. Nagashima T, Koga M, Odaka M, Hirata K, Yuki N. Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Arch Neurol. 2007 Oct;64(10):1519–23. Lardone RD, Yuki N, Odaka M, Daniotti JL, Irazoqui FJ, Nores GA. Anti-GM1 IgG antibodies in Guillain-Barré syndrome: fine specificity is associated with disease severity. J Neurol Neurosurg Psychiatry. 2010 Jun;81(6):629–33. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 23 Oct, 2024 Read the published version in BMC Neurology → Version 1 posted Editorial decision: Revision requested 07 Jun, 2024 Reviewers agreed at journal 04 Jun, 2024 Reviewers agreed at journal 04 Jun, 2024 Reviews received at journal 04 Jun, 2024 Reviewers agreed at journal 24 May, 2024 Reviews received at journal 13 May, 2024 Reviewers agreed at journal 13 May, 2024 Reviews received at journal 26 Apr, 2024 Reviewers agreed at journal 26 Apr, 2024 Reviewers invited by journal 12 Apr, 2024 Editor invited by journal 08 Apr, 2024 Submission checks completed at journal 03 Apr, 2024 Editor assigned by journal 03 Apr, 2024 First submitted to journal 03 Apr, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4211221","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":287372136,"identity":"e9c1d81f-848e-4b43-86e4-ba9eab41af57","order_by":0,"name":"Shota Ito","email":"","orcid":"","institution":"Nagoya University Graduate School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Shota","middleName":"","lastName":"Ito","suffix":""},{"id":287372139,"identity":"a063c86f-112b-40dd-a0c0-c843ae58e3cd","order_by":1,"name":"Satoshi Yokoi","email":"","orcid":"","institution":"Nagoya University Graduate School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Satoshi","middleName":"","lastName":"Yokoi","suffix":""},{"id":287372142,"identity":"34736ea9-bc64-480a-b679-fa3151f42146","order_by":2,"name":"Yuki Fukami","email":"","orcid":"","institution":"Nagoya University Graduate School of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Yuki","middleName":"","lastName":"Fukami","suffix":""},{"id":287372144,"identity":"adef7514-aafd-4f48-b9b9-7b33fdeb53ae","order_by":3,"name":"Ayumi Uchibori","email":"","orcid":"","institution":"Kyorin University","correspondingAuthor":false,"prefix":"","firstName":"Ayumi","middleName":"","lastName":"Uchibori","suffix":""},{"id":287372147,"identity":"5601aca5-aaaa-4d71-9a11-4e5f1c31a25f","order_by":4,"name":"Masahisa Katsuno","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABFUlEQVRIie2QP0vEMBiH31KIS1rXhMjdV0gpFOQQv0pKh9t0cOlwaEGoSz/AHYifwamrkUBdCq4nWbzF+QoihVsuN4iIpOommIcMP3jz8P4BcDj+IAR8CZCbJD8X0ICCBED7OwVz8Mqvih16JZPn7qYehTK4715zNQYivHUP4YlNYVhMo0WtYyrDjB20KiqI8GkF6MymjEA0LKh1eisxZ7RU3t1SADO7pIVN2V+VLLjWF0aJN0Y53nXZDCmMZIgFhRZc4oR2pUqNgga70PkLovNGRwuFkwm006zAq/Kw4vZdyOPpG1nP9Dh8qOKnPp8cFXuZWvZ5Y73YB755GC5N8sxIvBHfK7u/PZy/59nPFIfD4fgPbAGYJ1XjgICKtQAAAABJRU5ErkJggg==","orcid":"","institution":"Nagoya University Graduate School of Medicine","correspondingAuthor":true,"prefix":"","firstName":"Masahisa","middleName":"","lastName":"Katsuno","suffix":""}],"badges":[],"createdAt":"2024-04-03 08:37:37","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4211221/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4211221/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12883-024-03899-3","type":"published","date":"2024-10-23T15:58:21+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":54315318,"identity":"76141e80-93a8-4104-bac0-3218f783478d","added_by":"auto","created_at":"2024-04-08 17:43:52","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":909479,"visible":true,"origin":"","legend":"\u003cp\u003eNeurological examination of finger extension.\u003c/p\u003e\n\u003cp\u003ea: The patient exhibited the finger drop sign on the day of admission. b: The finger drop sign showed improvement on the 17th day after starting IVIg treatment.\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-4211221/v1/c40912729d0f3fae3c26c967.png"},{"id":54315316,"identity":"7ca61dff-183c-438a-a734-b13f2d944196","added_by":"auto","created_at":"2024-04-08 17:43:52","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":39191,"visible":true,"origin":"","legend":"\u003cp\u003eRight median nerve conduction tracings on day 1 and day 21 after admission.\u003c/p\u003e\n\u003cp\u003eAlthough the findings on the first day suggested conduction block, there was an improvement in the amplitude by the 21st day.\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-4211221/v1/c27bf855ae182c03878175a1.png"},{"id":54315320,"identity":"2bbcfb1b-2375-411b-ac50-b4e17117b145","added_by":"auto","created_at":"2024-04-08 17:43:52","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":117362,"visible":true,"origin":"","legend":"\u003cp\u003eThe time course of manual muscle testing, %vital capacity (VC), and tongue strength.\u003c/p\u003e\n\u003cp\u003eThe graph shows the time course of manual muscle testing, %VC, and tongue\u003c/p\u003e\n\u003cp\u003estrength relative to the average values of a 70-year-old population. All measurements gradually\u003c/p\u003e\n\u003cp\u003eimproved following IVIg treatment. The improvement in finger extension was slower than that of\u003c/p\u003e\n\u003cp\u003eflexion because of the high severity at onset.\u003c/p\u003e","description":"","filename":"Figure3.png","url":"https://assets-eu.researchsquare.com/files/rs-4211221/v1/d6b88bf4efea6ab9d7ea3cc6.png"},{"id":67682084,"identity":"6e76ef3f-5614-423a-b8fa-56ec1056cf7f","added_by":"auto","created_at":"2024-10-28 16:13:12","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1746183,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4211221/v1/e78444ce-a8bc-4e5c-9de9-48391348cea1.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Guillain-Barré syndrome with overlap between the finger drop variant and acute bulbar palsy: a case report","fulltext":[{"header":"Background","content":"\u003cp\u003eGuillain-Barr\u0026eacute; syndrome (GBS) is an acute autoimmune disorder that predominantly affects the peripheral nervous system and is characterized by a rapidly progressing course. Classical GBS typically begins with weakness in the legs that progressively ascends to involve the upper limbs, and in more severe cases, the respiratory muscles and cranial nerves (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eGBS is characterized by a variety of subtypes, each defined by specific clinical features, a wide variety of which have been highlighted over time. For instance, the \u0026lsquo;finger drop sign\u0026rsquo; was recently identified as a variant of GBS, known as the finger drop variant (FDv) (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). It is defined by prominent distal weakness in the upper limbs, predominantly affecting the fingers or wrist extensors more than the flexors. This variant usually exhibits no weakness or only mild weakness in the lower limbs. Because of its focal weakness and low disease frequency, this variant poses diagnostic challenges.\u003c/p\u003e \u003cp\u003eAnother important clinical symptom in patients with GBS is acute bulbar palsy. This symptom is typical of the pharyngeal-cervical-brachial (PCB) variant, which is typified by oropharyngeal and cervicobrachial weakness, without lower limb involvement (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). However, some patients with GBS primarily present with acute bulbar palsy without significant neck and brachial weakness, indicating another GBS variant (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). Such variable manifestation often complicates the diagnostic process, especially in the early stage. Furthermore, overlap between these variants sometimes occurs in patients with GBS, and this can make the diagnosis extremely complicated for clinicians. However, anti-ganglioside antibodies may help elucidate the clinical subtypes (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). It was reported that IgG anti-GM1 antibodies, as well as the ganglioside complex of anti-GM1 antibodies with phosphatidic acid, are linked to typical GBS and FDv (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). Similarly, anti-GD1b antibodies are commonly associated with sensory ataxic neuropathy, whereas anti-GT1a antibodies are related to PCB variants and acute bulbar palsy in patients with GBS (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). Furthermore, anti-GQ1b antibodies are often associated with Miller Fisher syndrome (MFS) (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). While definitive associations between these variants and specific anti-ganglioside antibodies have not been firmly established, the presence of these antibodies is often informative for the diagnosis of GBS.\u003c/p\u003e \u003cp\u003eIn this report, we present a case of a patient with GBS who exhibited a combination of quite atypical features, specifically, the finger drop sign and acute bulbar palsy due to tongue weakness. A precise neurological examination was important for the accurate diagnosis of this case in the early stage. Anti-ganglioside antibodies were useful to explain the phenotypes.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eAn 81-year-old man with a history of appendix cancer that recurred following treatment with cetuximab and encorafenib was admitted to a neurology department because of dysarthria, dysphagia, and weakness of the upper limbs lasting for 3 days. The patient had received no recent vaccinations, and had no prior infection, diarrhea, or upper respiratory symptoms. Neurological examination of the cranial nerves revealed dysarthria and dysphagia with an inability to protrude the tongue beyond the dental arch. There were no abnormalities in ocular movements and facial strength. The gait was normal, and no ataxia was observed; additionally, touch sensation and proprioception were also normal. Manual muscle testing conducted using the Medical Research Council scale revealed muscle weakness in the distal upper limbs. Bilateral wrist extensors and flexors scored two on the right and three on the left. Finger extensors were notably weak, with a uniform rating of two bilaterally, and the finger drop sign being shown (Fig. \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003ea). By contrast, the finger flexors exhibited only mild weakness, rated at three on the right and four on the left. The iliopsoas and hamstring muscles showed mild weakness, each scoring four. Muscles in other areas of the lower limbs were found to be at full strength. Tendon reflexes were diminished in the brachioradialis and triceps, but were normal in other sites. The blood test results were normal with no anti-SSA antibody, anti-SSB antibody, myeloperoxidase and proteinase 3 anti-neutrophil cytoplasmic antibodies, anti-acetylcholine receptor antibody, or anti-muscle-specific tyrosine kinase antibody. Nerve conduction studies revealed low amplitudes and deteriorated velocities with proximal stimulation in the median and ulnar motor nerves, with the results otherwise being within normal limits (Table \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e and Fig. \u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e). Respiratory function tests demonstrated a vital capacity of 71%. A cerebral spinal fluid test showed elevated protein levels of 48 mg/dL and normal cell counts, with five cells per mm\u0026sup3;. Brain MRI and cervical contrast-enhanced MRI were normal. Tongue pressure quantified with a balloon-type tongue pressure measurement device (TPM-02; JMS Co. Ltd, Hiroshima, Japan) was low, at an average of 10.44 kPa over ten trials, with this being only 33% of the average of 32 kPa for a 70-year-old population (\u003cspan class=\"CitationRef\"\u003e5\u003c/span\u003e). Videoendoscopic evaluation of swallowing revealed reduced pharyngeal contractility and normal laryngeal function.\u003c/p\u003e\n\u003cp\u003e\u003c/p\u003e\u0026nbsp;\u003ctable id=\"Tab1\" border=\"1\"\u003e\n \u003ccaption language=\"En\"\u003e\n \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\n \u003cdiv class=\"CaptionContent\"\u003e\n \u003cp\u003eThe results of nerve conduction studies on day 1 and 21 after admission\u003c/p\u003e\n \u003c/div\u003e\n \u003c/caption\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003cth align=\"left\" style=\"width: 6.7566%;\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 7.4857%;\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 7.1941%;\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003eday 1\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 7.1941%;\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003eday 21\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\n \u003cp\u003eNerve\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eStimulation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003eLatency(ms)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003eCMAP amp(mV)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003eVelocity(m/s)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003eLatency(ms)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003eCMAP amp(mV)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003eVelocity(m/s)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\n \u003cp\u003eRt. median\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eWrist\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e3.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e6.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e3.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e6.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eElbow\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e7.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e2.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e47.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e8.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e3.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e47.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\n \u003cp\u003eRt. ulnar\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eWrist\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e2.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e7.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e2.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e7.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eBelow elbow\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e7.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e4.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e53.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e7.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e6.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e48.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eAbove elbow\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e10.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e4.6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e42.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e9.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e6.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e47.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\n \u003cp\u003eRt. peroneal\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eAnkle\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e6.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e1.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e5.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e3.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eFibular head\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e15.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e1.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e40.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e14.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e1.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e39.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\n \u003cp\u003eRt. tibial\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003eAnkle\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e4.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e8.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e3.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e9.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" style=\"width: 6.7566%;\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.4857%;\"\u003e\n \u003cp\u003ePop fossa\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e14.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e4.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e41.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.1941%;\"\u003e\n \u003cp\u003e14.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 9.3328%;\"\u003e\n \u003cp\u003e5.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\" style=\"width: 7.7774%;\"\u003e\n \u003cp\u003e41.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003ctfoot\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"8\" style=\"width: 63.58%;\"\u003eOn the first day, the study revealed low amplitude and reduced conduction velocities in the proximal segments of the median and ulnar motor nerves. By the 21st day, there was a slight improvement in the amplitude of these nerves.\u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tfoot\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003c/p\u003e\n\u003cp\u003eAs a result, we strongly suspected GBS because of the acute onset of the mostly symmetric pattern of limb and cranial nerve weakness with areflexia and albuminocytologic dissociation. Consequently, intravenous immunoglobulin therapy (0.4 g/day for 5 consecutive days), rehabilitation, and fluid administration were initiated on the day following admission. After the treatment, the patient\u0026rsquo;s symptoms gradually improved. By the 17th day of admission, there was a gradual improvement in the finger drop sign (Fig. \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003eb), and the patient was able to resume normal oral intake. The improvement in finger extension was slower than that of flexion, in line with the severity at onset. The patient\u0026rsquo;s tongue pressure had improved to 23.56 kPa, 74% relative to the normal value for a 70-year-old population (Fig. \u003cspan class=\"InternalRef\"\u003e3\u003c/span\u003e). On the 21st day of admission, we again conducted nerve conduction studies (Table \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e and Fig. \u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e), which showed a slight recovery in amplitude and a mild decrease in velocities, in line with acute motor axonal neuropathy (AMAN) (\u003cspan class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan class=\"CitationRef\"\u003e8\u003c/span\u003e). An enzyme-linked immunosorbent assay (ELISA) of serum IgG antibodies to gangliosides revealed positive results for GM1, asialo-GM1, GD1b, GT1a, and GQ1b, with titers of 0.422, 0.230, 0.404, and 0.452, respectively. On the 31st day after admission, the patient was discharged from the hospital. Given the monophasic disease course and the results of the nerve conduction studies, a diagnosis of AMAN, a subtype of GBS, was established.\u003c/p\u003e"},{"header":"Discussion and Conclusion","content":"\u003cp\u003eThe patient presented with the finger drop sign and acute bulbar palsy, which are both relatively rare manifestations of GBS. First, the finger drop sign is a distinctive feature observed in this case. Recent research defined the FDv as corresponding to the AMAN type in nerve conduction studies (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). Initially, our patient\u0026rsquo;s nerve conduction studies demonstrated low amplitude, suggesting a conduction block. However, early reversible conduction failure is a known phenomenon in GBS (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e), with this abnormality often being observed in the early stage of AMAN type. In subsequent nerve conduction studies, the conduction velocities returned to normal, confirming the AMAN classification of our case according to the Ho criteria, Hadden criteria, and Uncini criteria (\u003cspan additionalcitationids=\"CR7\" citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e). While our case fulfills the definition of FDv, including positive anti-GM1 antibodies and AMAN type, it is important to note that the presence of a combination of positive anti-GT1a antibodies in FDv has not been previously reported.\u003c/p\u003e \u003cp\u003eThe occurrence of acute bulbar palsy in GBS is frequently associated with anti-GT1a antibodies (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e). Interestingly, there are no specific studies that have conclusively identified which cranial nerves are predominantly involved in acute bulbar palsy in patients with GBS with positive anti-GT1a antibodies (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). Our patient exhibited pronounced tongue weakness at the onset of GBS, which improved rapidly following IVIg treatment. The improvement in tongue pressure was associated with amelioration of the dysarthria and dysphasia, suggesting that tongue pressure may reflect the overall severity of GBS. Tongue weakness is reported to be correlated with respiratory function in GBS (\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e). As GT1a is reported to be expressed in the glossopharyngeal nerve, vagal nerve, human cerebrum, and hypoglossal nerve, the presence of anti-GT1a antibodies raises the possibility that the effect of these antibodies on the hypoglossal nerve could cause the unique tongue weakness (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). Measuring tongue strength can be a valuable tool to not only assess the severity and progression of bulbar palsy, but to also determine the targets of intervention in rehabilitation.\u003c/p\u003e \u003cp\u003eIn our case, anti-GD1b and anti-GQ1b antibodies were also positive. While anti-GD1b antibodies indicate sensory ataxic neuropathy and anti-GQ1b antibodies are typically indicative of MFS, the clinical presentation of our patient did not exhibit ataxia and did not align with MFS (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e). Despite the occurrence of anti-GD1b and GQ1b antibodies in MFS and sensory ataxic neuropathy cases, it is important to consider the possibility of cross reactivity (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e). The absence of typical MFS symptoms and reduced deep sensation indicated that the presence of anti-GD1b and anti-GQ1b antibodies in this case may have been due to cross reactivity to anti-GM1 and anti-GT1a antibodies, respectively. In addition, while the patient had appendix cancer, there was low probability of paraneoplastic syndrome because of his monophasic disease course and marked response to IVIg treatments.\u003c/p\u003e \u003cp\u003eOur case exhibited a rare combination of clinical features, namely, acute bulbar palsy and the finger drop sign. The simultaneous presence of these symptoms, coupled with the detection of both anti-GT1a and anti-GM1 antibodies, highlights the intricacies and diagnostic challenges associated with overlapping subtypes of GBS, underscoring the need for clinicians to consider the possibility of such overlapping syndromes. Our case particularly suggests an association between anti-GT1a antibody and its impact on the hypoglossal nerve. This finding suggests that measuring tongue strength could be a valuable method for assessing clinical progression and determining appropriate rehabilitation strategies. To disentangle the complexities of these overlapping subtypes, further research is essential to elucidate the distinct clinical subtypes of GBS, their specific associations with anti-ganglioside antibodies, and the mechanisms underlying these associations.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eGBS: Guillain-Barr\u0026eacute; syndrome\u003c/p\u003e\n\u003cp\u003eFDv: finger drop variant\u003c/p\u003e\n\u003cp\u003ePCB: Pharyngeal-cervical-brachial\u003c/p\u003e\n\u003cp\u003eMFS: Miller Fisher syndrome\u003c/p\u003e\n\u003cp\u003eMRI: Magnetic resonance imaging\u003c/p\u003e\n\u003cp\u003eIVIg: Intravenous immunoglobulin\u003c/p\u003e\n\u003cp\u003eELISA: Enzyme-linked immunosorbent assay\u003c/p\u003e\n\u003cp\u003eAMAN: Acute motor axonal neuropathy\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eAcknowledgments:\u003c/p\u003e\n\u003cp\u003eNot applicable.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eFunding:\u003c/p\u003e\n\u003cp\u003eNone.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAvailability of data and materials:\u003c/p\u003e\n\u003cp\u003eAll data analyzed are included in the published article.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAuthor\u0026rsquo;s contributions:\u003c/p\u003e\n\u003cp\u003eShota Ito, Satoshi Yokoi, and Yuki Fukami drafted the manuscript. Ayumi Uchibori measured serum IgG antibodies against gangliosides. Masahisa Katsuno reviewed and revised the manuscript. All authors read and approved the final manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eEthics approval and consent to participate:\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eConsent for publication:\u003c/p\u003e\n\u003cp\u003eThe authors have obtained written informed consent from the patient for the publication of this paper.\u003c/p\u003e\n\u003cp\u003eCompeting interests:\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eWakerley BR, Uncini A, Yuki N, GBS Classification Group, GBS Classification Group. Guillain-Barr\u0026eacute; and Miller Fisher syndromes--new diagnostic classification. Nat Rev Neurol. 2014 Sep;10(9):537\u0026ndash;44.\u003c/li\u003e\n\u003cli\u003eYoon B-A, Ha D-H, Park HT, Kusunoki S, Kuwahara M, Lee JH, et al. Finger drop sign as a new variant of acute motor axonal neuropathy. Muscle Nerve. 2021 Mar;63(3):336\u0026ndash;43.\u003c/li\u003e\n\u003cli\u003eWakerley BR, Yuki N. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry. 2014 Mar;85(3):339\u0026ndash;44.\u003c/li\u003e\n\u003cli\u003eKoga M, Yoshino H, Morimatsu M, Yuki N. Anti-GT1a IgG in Guillain-Barr\u0026eacute; syndrome. J Neurol Neurosurg Psychiatry. 2002 Jun;72(6):767\u0026ndash;71.\u003c/li\u003e\n\u003cli\u003eUtanohara Y, Hayashi R, Yoshikawa M, Yoshida M, Tsuga K, Akagawa Y. Standard values of maximum tongue pressure taken using newly developed disposable tongue pressure measurement device. Dysphagia. 2008 Sep;23(3):286\u0026ndash;90.\u003c/li\u003e\n\u003cli\u003eHo TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, et al. Guillain-Barr\u0026eacute; syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995 Jun 1;118 ( Pt 3)(3):597\u0026ndash;605.\u003c/li\u003e\n\u003cli\u003eHadden RD, Cornblath DR, Hughes RA, Zielasek J, Hartung HP, Toyka KV, et al. Electrophysiological classification of Guillain-Barr\u0026eacute; syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barr\u0026eacute; Syndrome Trial Group. Ann Neurol. 1998 Nov;44(5):780\u0026ndash;8.\u003c/li\u003e\n\u003cli\u003eUncini A, Kuwabara S. Electrodiagnostic criteria for Guillain-Barr\u0026egrave; syndrome: a critical revision and the need for an update. Clin Neurophysiol. 2012 Aug;123(8):1487\u0026ndash;95.\u003c/li\u003e\n\u003cli\u003eChan Y-C, Punzalan-Sotelo AM, Kannan TA, Shahrizaila N, Umapathi T, Goh EJH, et al. Electrodiagnosis of reversible conduction failure in Guillain-Barr\u0026eacute; syndrome. Muscle Nerve. 2017 Nov;56(5):919\u0026ndash;24.\u003c/li\u003e\n\u003cli\u003eKim JK, Kim B-J, Shin HY, Shin KJ, Nam T-S, Oh J, et al. Acute bulbar palsy as a variant of Guillain-Barr\u0026eacute; syndrome. Neurology. 2016 Feb 23;86(8):742\u0026ndash;7.\u003c/li\u003e\n\u003cli\u003eGarg N, Yuki N, Park SB, Barnett MH, Kiernan MC. Acute bulbar, neck and limb weakness with monospecific anti‐GT1a antibody: A rare localized subtype of Guillain‐Barr\u0026eacute; sydnrome. Muscle Nerve. 2016 Jan;53(1):143\u0026ndash;6.\u003c/li\u003e\n\u003cli\u003eOrlikowski D, Terzi N, Blumen M, Sharshar T, Raphael JC, Annane D, et al. Tongue weakness is associated with respiratory failure in patients with severe Guillain-Barr\u0026eacute; syndrome. Acta Neurol Scand. 2009 Jun;119(6):364\u0026ndash;70.\u003c/li\u003e\n\u003cli\u003eYoshino H, Harukawa H, Asano A. IgG antiganglioside antibodies in Guillain\u0026ndash;Barre syndrome with bulbar palsy. J Neuroimmunol. 2000, Jun 26;105(2):195\u0026ndash;201.\u003c/li\u003e\n\u003cli\u003eSusuki K, Yuki N, Hirata K. Fine specificity of anti-GQ1b IgG and clinical features. J Neurol Sci. 2001 Mar 15;185(1):5\u0026ndash;9.\u003c/li\u003e\n\u003cli\u003eNagashima T, Koga M, Odaka M, Hirata K, Yuki N. Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barr\u0026eacute; syndrome. Arch Neurol. 2007 Oct;64(10):1519\u0026ndash;23.\u003c/li\u003e\n\u003cli\u003eLardone RD, Yuki N, Odaka M, Daniotti JL, Irazoqui FJ, Nores GA. Anti-GM1 IgG antibodies in Guillain-Barr\u0026eacute; syndrome: fine specificity is associated with disease severity. J Neurol Neurosurg Psychiatry. 2010 Jun;81(6):629\u0026ndash;33.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Guillain-Barré syndrome, finger drop sign, bulbar palsy, anti-ganglioside antibody","lastPublishedDoi":"10.21203/rs.3.rs-4211221/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4211221/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eGuillain-Barr\u0026eacute; syndrome (GBS) encompasses diverse variants, and the overlap between these variants often makes the diagnosis of GBS difficult. We report a case of GBS overlapping with both the finger drop variant and acute bulbar palsy.\u003c/p\u003e\u003ch2\u003eCase presentation:\u003c/h2\u003e \u003cp\u003eAn 81-year-old man presented with dysarthria, dysphagia, and upper limb weakness. The patient exhibited impaired tongue protrusion and the finger drop sign, whereas other cranial nerve functions, leg strength, touch sensation, and proprioception were normal. Brachioradialis and triceps reflexes were diminished. Cerebrospinal fluid analysis revealed elevated protein levels with normal cell counts. Nerve conduction studies showed reduced amplitudes and decreased velocities in the median and ulnar nerves. As GBS was suspected, we initiated intravenous immunoglobulin treatment, resulting in gradual improvement within 3 weeks. Serum IgG antibodies to gangliosides tested positive for GM1, asialo-GM1, GT1a, GD1b, and GQ1b. On the basis of the monophasic disease course and anti-ganglioside antibodies, we confirmed the diagnosis of GBS.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eThis case is the first reported instance of an overlap between the finger drop variant and acute bulbar palsy in GBS, highlighting the importance of considering GBS when patients present with a combination of atypical symptoms. Anti-ganglioside antibodies may assist in distinguishing these complex symptoms.\u003c/p\u003e","manuscriptTitle":"Guillain-Barré syndrome with overlap between the finger drop variant and acute bulbar palsy: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-04-08 17:43:47","doi":"10.21203/rs.3.rs-4211221/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-06-07T10:18:48+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"238842264599145445984437111681575763916","date":"2024-06-04T19:55:34+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"139735788312602840697445698201430386624","date":"2024-06-04T17:27:51+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-06-04T15:03:23+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"306570946175352969495547171378005726903","date":"2024-05-24T05:22:57+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-14T02:02:05+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"26968883139125947173595707430121245893","date":"2024-05-13T08:31:35+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-04-27T01:57:26+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"6801932486875977820049771260832653223","date":"2024-04-27T01:17:07+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-04-12T23:30:28+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2024-04-08T13:03:31+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-04-04T00:02:45+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-04-04T00:02:45+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Neurology","date":"2024-04-03T08:36:18+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"313d06f5-b645-4f61-bd0f-6054b15de921","owner":[],"postedDate":"April 8th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-10-28T16:06:39+00:00","versionOfRecord":{"articleIdentity":"rs-4211221","link":"https://doi.org/10.1186/s12883-024-03899-3","journal":{"identity":"bmc-neurology","isVorOnly":false,"title":"BMC Neurology"},"publishedOn":"2024-10-23 15:58:21","publishedOnDateReadable":"October 23rd, 2024"},"versionCreatedAt":"2024-04-08 17:43:47","video":"","vorDoi":"10.1186/s12883-024-03899-3","vorDoiUrl":"https://doi.org/10.1186/s12883-024-03899-3","workflowStages":[]},"version":"v1","identity":"rs-4211221","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4211221","identity":"rs-4211221","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}