The Case Files

Abstract

A patient presented with facial droop and diplopia soon after dental workFigureFigureFigureFigureA 65-year-old woman with a history of essential hypertension, sinus bradycardia, hyperlipidemia, and hypothyroidism presented after one day of sudden-onset left-sided facial droop, left ptosis with diplopia, and dizziness. She had had a constant, severe sore throat, dysphagia, dry cough, nausea, and vomiting for four days following a dental procedure before these symptoms started. The patient's husband reported that she was unable to tolerate oral intake since the procedure. He brought her to the emergency department because of her persistent left-sided facial droop and vomiting. The patient was hypertensive with a blood pressure of 213/83 mm Hg; all other vital signs were normal. Her labs were remarkable for heme concentration with a hemoglobin of 16 gm/dL and hematocrit of 46.5%. An exam revealed left facial droop with forehead involvement, left ptosis, bilateral cranial nerve XI palsy, bilateral patellar areflexia, muffled voice, and drooling with an inability to elevate her tongue. The patient had no recent head trauma nor a family history of autoimmune diseases. The patient's neuromuscular symptoms gradually worsened. She developed a horizontal and vertical gaze nystagmus (left greater than right), with new onset of right-sided ptosis. She had an extensive workup using the MRI stroke protocol, which was normal, and CT angiogram ruled out dissection. Multiple serological studies were ordered, including AChR and anti-MuSK antibodies, both of which were unremarkable. Her neurological symptoms gradually improved after receiving plasmapheresis (PLEX) for five days and intravenous immunoglobulin (IVIG) for three days. The Infection Connection The patient's symptoms mainly involved the cranial nerves, and were suggestive of Miller Fisher syndrome (MFS), a rare, atypical variant of Guillain-Barre syndrome (GBS). MFS occurs in one to five percent of cases of GBS in Western countries, 25 percent in Japan, and 19 percent in Taiwan. MFS is 60 to 68 percent more prevalent in male than female patients. (J Neuroophthalmol. 2009;29[4]:312; https://bit.ly/3VoruAI.) GBS is an acute idiopathic neurological disease that most commonly presents with symmetrical paralysis of bilateral lower extremities due to its demyelinating process of the peripheral nerves and axonal involvement, which progresses in an ascending manner. (J Can Chiropr Assoc. 1995;39[2]:80; https://bit.ly/3LMuWlt.) MFS is an acute idiopathic polyneuropathy disease that is diagnosed clinically characterized by the triad of ophthalmoplegia, areflexia, and ataxia and that progresses in descending fashion. (J Neurol Neurosurg Psychiatry. 2002;72[5]:680; https://bit.ly/410twYS.) The etiologies of GBS and MFS are unknown given their idiopathic nature, but it is believed that these neurological conditions present following an upper respiratory infection or gastrointestinal infection, most commonly Campylobacter jejuni and Haemophilus influenza. (Hawaii J Med Public Health. 2016;75[7]:196; https://bit.ly/3HviPqq.) Roughly 18 percent of MFS cases and 31 percent of GBS cases are preceded by C. jejuni infection. (Arch Neurol. 2004;61[7]:1013; https://bit.ly/44jO3dM.) MFS gets its name from Charles Miller Fisher, MD, a Canadian neurologist who discovered three patients who shared its triad characteristics in 1959. (StatPearls [Internet]. June 2022; https://bit.ly/3Hwfk2O.) A Japanese study of 50 MFS cases indicated that 78 percent presented with diplopia, 46 percent with ataxia, and 34 percent with diplopia and ataxia. (Hawaii J Med Public Health. 2016;75[7]:196; https://bit.ly/3HviPqq; J Neuroophthalmol. 2009;29[4]:312; https://bit.ly/3VoruAI.) Another study discovered that the serum IgG antibody to the GQ1b ganglioside was prevalent in approximately 90 percent of MFS cases. This serological study alone is not diagnostic due to other neurological conditions, such as Bickerstaff brainstem encephalitis and GBS, that contain the anti-GQ1b IgG antibody. (Hawaii J Med Public Health. 2016;75[7]:196; https://bit.ly/3HviPqq; Arch Neurol. 2004;61[7]:1013; https://bit.ly/44jO3dM.) A diagnosis can be made with exam findings and a positive GQ1b antibody serum study. A differential diagnosis without known MFS should include pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, myasthenia gravis, Horner syndrome, Bickerstaff brainstem encephalitis, and an intracranial mass. (Cureus. 2017;9[2]:e1048; https://bit.ly/3ngV5Q6; Arch Neurol. 2004;61[7]:1013; https://bit.ly/44jO3dM.) IVIG and PLEX The most efficacious treatments for patients with MFS and GBS are intravenous IVIG and PLEX, which have been proven to slow the progression of the disease process and gradually improve the patient's neurological symptoms. The patient's prognosis and duration for recovery is dependent on the severity of the symptoms with which he initially presented and the time since the onset of symptoms before presentation. The duration of recovery is not affected by treatments received, nor are the treatments invasive or harmful. (StatPearls [Internet]. June 2022; https://bit.ly/3Hwfk2O.) Multiple studies have evaluated the efficacy of treatments for MFS and GBS. Treatment of MFS with corticosteroids alone in six clinical trials with 587 participants showed no significant improvement in symptoms or the progression of neurological symptoms after four weeks, although corticosteroids have been proven effective in addition to IVIG and PLEX. (Cochrane Database Syst Rev. 2016;[10]:CD001446; https://bit.ly/41WBBiz.) A study comparing IVIG and corticosteroids with IVIG alone showed 76 percent of patients had symptom improvement with steroids and IVIG in comparison with 56 percent of patients on IVIG alone. (NORD. March 30, 2020; https://bit.ly/3oYHLQI.) The typical duration of recovery for MFS is two to four months, with a fatality rate of less than five percent. GBS also has a five percent fatality rate, and roughly 10 percent of patients are left with chronic disabilities. (StatPearls [Internet]. June 2022; https://bit.ly/3Hwfk2O.) Our patient had extensive brain imaging to rule out cerebral vascular accident. All imaging was negative for acute intracranial hemorrhage and intracranial masses. Neurology evaluated her extensively, and she was initially thought to have myasthenia gravis with bulbar symptoms, but her AChR and anti-MuSK antibodies were unremarkable. Analysis of the cerebrospinal fluid was unremarkable for an abnormal protein or glucose level, which is common in patients with GBS. Serological studies of the human simplex virus and polymerase chain reaction also yielded unremarkable results. She was diagnosed with MFS after a positive GQ1b IgG antibody serum study. She was empirically started on vancomycin and piperacillin/tazobactam for broad-spectrum coverage of possible encephalitis/meningitis, acyclovir, and prednisone. She was emergently intubated for airway protection secondary to the inability to tolerate oral secretions because of concerns for aspiration. She was treated with IVIG for three days, prednisone for five days, and PLEX for five days. A gastrostomy-jejunostomy tube was inserted for nutritional supplementation because of her dysphagia, and she had a tracheostomy for hypoxic respiratory failure. She was admitted to acute rehabilitation and had extensive speech, occupational, and physical therapy. She has a good prognosis, but is likely to be tracheostomy-dependent. Share this article on Twitter and Facebook. Access the links in EMN by reading this on our website: www.EM-News.com. Comments? Write to us at [email protected].