Paralytic Ileus as First Symptom of Miller Fisher Syndrome: a Case Report

doi:10.21203/rs.3.rs-1452158/v1

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Case Report

Paralytic Ileus as First Symptom of Miller Fisher Syndrome: a Case Report

https://doi.org/10.21203/rs.3.rs-1452158/v1

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Introduction Miller Fisher syndrome (MFS) is a nervous disease, immune correlation of Guillain Barre syndrome (GBS).The typical clinical manifestations of MFS are extraocular muscle paralysis, ataxia, tendon reflex loss or disappearance. This report describes a case of MFS with paralytic ileus as the first symptom.

Case presentation A 48-year-old woman presenting with abdominal pain and distention was diagnosed with paralytic ileus. There was no significant improvement in symptoms after symptomatic treatment. After that, the patient developed visual rotation, with limited binocular abduction and adduction, and ataxia. Anti-ganglioside testing revealed positive anti-GQ1b antibodies. The patient was diagnosed as Miller Fisher syndrome. And recovered after symptomatic treatment.

Conclusion In case of inconsistency between imaging examination and clinical symptoms, neuroelectrophysiology and cerebrospinal fluid puncture should be performed in time, and strive for timely detection and treatment.

Paralytic Ileus

Miller Fisher Syndrome

case report

Miller Fisher syndrome (MFS), regarded by many scholars as a variant of Guillain Barre syndrome (GBS) [1–2], accounts for about 5%-10% of GBS[3]. The typical clinical manifestations of MFS are extraocular muscle paralysis, ataxia, tendon reflex loss or disappearance. Other cases with dysphagia, photophobia, taste loss[4], limb numbness, facial paralysis[5] as initial cases were also reported. At present, intestinal obstruction is rarely as initial case reported.

A 48-year-old female was admitted to hospital with abdominal pain and abdominal distension, accompanied by cessation of exhaust and defecation for 2 weeks. At the time of admission, the patient had abdominal pain and distension with cessation of exhaust and defecation, fatigue, cough and expectoration, no nausea and vomiting, no dizziness and headache, and no blurred vision.

She had self-administered oral Chinese patent medicine for a cold within a week prior to her admission to the hospital. The patient has no history of chronic diseases such as hypertension or diabetes. She also had no history of fever or specific infections in the month before the disease and no family history of genetic predisposition.The patient denied alcohol consumption and allergies to food or medicines.

Physical examination on admission: yellowish complexion, clear-consciousness, fair-spirited, bilateral pupils equal in size and round, sensitive to light reflection, no obvious abnormalities in cardiopulmonary auscultation, abdomen distended, slightly tight, mild tenderness in the right abdomen and around the umbilicus, liver and spleen was not felt, bowel sounds weakened, and normal muscle strength of both lower limbs.

Laboratory examination: The patient’s laboratory test results are shown in Table 1.

Table 1

Laboratory test results
Blood tests	Result	Reference values
Red blood cells	3.56×10¹²/L	3.80–5.10×10¹²/L
Hemoglobin	100 g / L	115–150 g / L
Albumin	29.1 g / L	44.0–55.0 g / L
Potassium	3.79 mmol / L	3.50–5.30 mmol / L
Calcium	2.03 mmol / L	2.11–2.52 mmol / L
Sodium	138 mmol / L	137–147 mmol / L

Imaging examinations: The patient’s Imaging examinations are shown in Fig. 1.

After admission, the patient was treated with fasting, continuous gastrointestinal decompression, nutritional support, correction of electrolyte disorders, inhibition of gastrointestinal gland secretion, and enema of hospital self-made cleansing compound. The patient’s intestinal obstruction symptoms were slightly improved after the treatment. 5 days after admission, the patient had nausea, eating choking cough, and accompanied by dizziness, visual rotation. Brain MRI were shown in Fig. 2

After symptomatic treatment, the patient’s symptoms did not significantly improve, and the visual object was further re-shadowed, binoculus abducted and adduction was limited. At this time, physical examination was performed: the patient was clear-consciousness, poor spirit, clear and fluent in speech, active position, and cooperative in physical examination, no enlargement or narrowing of the palpebral fissure, binoculus abducted and adduction was limited. There were vertical nystagmus when the eyes were up-looking. The muscle strength and muscle tension of the extremities were normal, and the tendon reflex of the extremities was weakened. Extremities have deep and shallow sensations, ataxia occurred, and the pathological signs were not extracted. The patient’s abdomen distended, and there was tenderness around the umbilicus. Related laboratory tests are shown in Table 2.

Table 2

Laboratory test results
Laboratory tests	Result
Anti-GM1 antibody	Positive
Anti-GQ1b IgG antibody	Positive
Anti- GT1a IgG antibody	Positive

Visual evoked potential showed: VEP: bilateral P100 waveform differentiation is acceptable, repeatability is acceptable, the incubation period is normal. Cerebrospinal fluid examination : pressure : 200mmHg, protein quantification : 190mg / dL, white blood cell count 4×10⁶/L, Glucose and chloride were normal, and no obvious abnormality was found by electromyogram. The patient was diagnosed as Miller Fisher syndrome.

Then gave Hormone shock therapy: 250ml normal saline plus methylprednisolone 1000mg qD, methylprednisolone 500mg/d QD after 3d, methylprednisolone 250mg/d qD after 3d, 120mg/d qD after 3d, oral prednisone 60mg/d after 3d, Thereafter, reduce 5mg per week and gradually stop taking the medicine. Human immunoglobulin: immunoglobulin c was injected intravenously, and immunoglobulin c was injected 27.5g qd(0.4g/kg/d, patient weight 70kg) for consecutive 5 days. At the same time,gave nutrition nerve adjuvant therapy: Calcium carbonate 750mg tid, potassium citrate 10mL tid, famotidine 20mg bid to prevent hormonal side effects. For vitamin B1 20 mg tid. And paralytic ileus conventional treatment at the same time.

After 1 week, the symptoms of intestinal obstruction and MFS were gradually improved. The patient was later discharged automatically for financial reasons. 6 months after discharge, the patient was interviewed by telephone, and she was recovered.

The initial diagnosis of MFS is based on clinical core symptoms: tendon reflex weakening or disappearance, ataxia, and extraocular muscle paralysis, which need to be confirmed by clinical neurophysiology research and cerebrospinal fluid analysis[6]. At present, it is believed that 84% of MFS patients have a history of infection[7], and the pathogenesis is mainly related to the autoimmunity after infection. The antibodies produced after infection directly attack the presynaptic membrane and synaptic gap sites, resulting in the damage to peripheral nerve integrity and the loss of nerve conduction and other related functions[8]. It is generally believed that more than 90% of MFS patients are related to anti-GQ1b antibody, and anti-GD1b antibody, anti-GT1a antibody and anti-GM3 IgG antibody have also been reported[9]. It is generally assumed that MFS only involves the peripheral nervous system, and the central nervous system is not involved. However, with the in-depth study of MFS, it is found that the central nervous system of the disease can also be involved[10], and the brain stem and cerebellum are the most common sites of involvement[11].The specific pathological mechanism is still unclear, and some scholars believe that it may be consistent with the previous idea that immune mediation leads to the destruction of the blood-cerebrospinal fluid barrier[12].

The etiology of paralytic intestinal obstruction is generally a collective term for diseases with dilated intestines and loss of intestinal peristalsis due to various causes. The common causes include: (1) surgical stimulation. (2) Acute abdomen causes reflex paralytic ileus. (3) Electrolyte abnormalities. (4) Diseases such as intestinal obstruction. (5) Abdominal inflammatory stimulation. (6) Intestinal nerve diseases.

The patients were asked to have no recent surgical history, and there was no acute abdominal pain stimulation. Laboratory tests excluded severe infection and electrolyte abnormalities. Abdominal CT simply rule out abdominal organ lesions. Intestinal vascular embolism was excluded based on the patient’s symptoms, signs and CT examination. Patient had pre-infection history, progressive extraocular muscle paralysis, ataxia, medulla oblongata paralysis, respiratory depression, and laboratory tests were consistent with the diagnosis of MFS. The symptoms were relieved after applying large amounts of immunoglobulin and hormone shock therapy. Therefore, it is inferred that MFS is the cause of paralytic intestinal obstruction. At present, there are few MFS with intestinal obstruction as the initial symptom, but there are reports of GBS with intestinal obstruction as the initial symptom[13]. Studies have shown that about two-thirds of GBS patients have symptoms of autonomic nerve damage[14]. This includes blood pressure fluctuations, arrhythmias, vasomotor dysfunction, and gastrointestinal (GI) motility dysregulation. It is caused by infiltration of lymphocytes in the ganglion, hypothalamus and brainstem of the autonomic nerve, which destroys the intramural ganglion cells, causing inflammation and edema of the autonomic ganglion and leading to the lysis of nerve cells [15]. At the same time, Some reports suggest that there may be ganglioside autoantibodies, such as anti-gm1 antibodies [16], which can cause the excitation disorder of sympathetic and parasympathetic nervous system, leading to hyperfunction or hypofunction of sympathetic and parasympathetic nervous system[17]. The intimate supply of the GI tract with autonomic parasympathetic and sympathetic nerves can be targeted and then lead to intestinal obstruction. The cause of the first paralytic intestinal obstruction in this case may be related to the earliest involvement of the nerve dominating the intestine.

Ethics approval and consent to participate: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Consent for publication: Informed consent was obtained from the patient.

Availability of data and materials: Not applicable.

Competing interests: The authors declare that they have no competing interest.

Funding: Supported by Traditional Chinese Medicine Science and Technology Development Plan of Shandong Province,No.2019-0198

Acknowledgements: The authors would like to thank the patient and her family for providing consent to use her photograph/video in this article.

Authors' contributions: Xiubin Liu, Xiqi Chen, and Yongkun Zhou were the patient’s attending physicians, reviewed the literature, and contributed to manuscript drafting; Xiaoxia Zhang and Yongkun Zhou were responsible for the revision of the manuscript for important intellectual content; all authors issued final approval for the version to be submitted.

Wakerley BR, Uncini A, Yuki N, et al. Guillain-Barré and Miller Fisher syndromes-- new diagnostic classification[J]. Nat Rev Neurol, 2014, 10: 537-544.
Nat Rev Neurol, 2014, 10: 537-544. Teener JW. Miller Fisher's Syndrome[J]. Semin Neurol, 2012, 32: 512- 516.
van den Berg Bianca et al. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis.[J]. Nature reviews. Neurology, 2014, 10(8) : 469-82.
Yagi Y, Yokote H, Watanabe Y, et al. Taste impairment in Miller Fisher syndrome[J]. Neurol Sci, 2015, 36: 809-810.
Jung JH,Oh EH,Shin JH,et al.Atypical clinical manifestations of Miller Fisher syndrome[J].Neurol Sci,2019,40(1):67-73.
Bukhari S, Taboada J. A Case of Miller Fisher Syndrome and Literature Review[J]. Cureu, 2017, 9: e1048.
Tuzun E, Kurtuneu M, Lang B, et al. Biekerstaft’s encephalitis and Miller Fisher syndrome associated with voltage gated potassium channel and novel antineuronal antibodies[J]. Eur J Neuro, 2010, 17: 1304-1307.
Rodella U, Scorzeto M, Duregotti E, et al. An animal model of Miller Fisher syndrome: Mitochondrial hydrogen peroxide is produced by the autoimmune attack of nerve terminals and activates Schwann cells[J]. Neurobiology of Disease, 2016, 96: 95-104.
Matsuzono K,Ozawa T,Yoshikawa K,et al.A case report of Fisher syndrome with the detection of anti-GM3 and anti-GD1b IgG antibodies[J].Neurol Sci,2019,40(4):891-893.
Chikakiyo H , Kunishige M , Yoshino H , et al. Delayed motor and sensory neuropathy in a patient with brainstem encephalitis[J]. Journal of the Neurological Sciences, 2005, 234(1-2):105-108.
Teener JW.Miller Fisher’s syndrome[J].Semin Neurol,2012,32(5):512-516.
Segurado O G , H Krüger, Mertens H G . Clinical significance of serum and CSF findings in the Guillain-Barré syndrome and related disorders.[J]. Journal of Neurology, 1986, 233(4):202-208.
Adynamic Ileus in severe Guillain–Barré syndrome[J]. Muscle & Nerve, 2001, 24(7):963-965.
Ropper AH, Wijdicks EFM, Truax BT (1991) Guillain–Barré syndrome. Contemporary neurology series. FA Davis, Philadelphia.
Zochodne D W. Autonomic involvement in Guillain-Barré syndrome: a review.[J]. Muscle & Nerve, 1994, 17(10):1145-55.
Osler W. The principles and practice of medicine. NY D Appleton & Co.
Lichtenfeld P. Autonomic dysfunction in the Guillain-Barré syndrome[J]. American Journal of Medicine, 1971, 50(6):772-780.

No competing interests reported.

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Paralytic Ileus as First Symptom of Miller Fisher Syndrome: a Case Report

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