In an otherwise normal brain, GQ1b is found to be less prevalent in white matter to begin with, indicating that the presence of elevated levels on laboratory testing may hold significance (Marconi et?al

In an otherwise normal brain, GQ1b is found to be less prevalent in white matter to begin with, indicating that the presence of elevated levels on laboratory testing may hold significance (Marconi et?al., 2005). characterized by the triad of ataxia, ophthalmoplegia, and areflexia. While MFS and ALS are mainly diagnosed based on the clinical presentation of a patient, the role of ganglioside antibodies in the pathogenesis of these conditions has been studied in recent years. Gangliosides are sialic acid-containing glycosphingolipids that are present abundantly in peripheral nerves (GM1, GM2, GD1a, GD1b, GQ1b and Asialo-GM1). These antibodies are well known to be associated with GBS and MFS, with GQ1b particularly prevalent for MFS (81% prevalence) (Yuki et?al., 1993). Ganglioside antibodies have also been reported in some patients with ALS. When an antibody is present, it is usually GM1 (Pestronk et?al., 1989, 1998; Lamb and Patten, 1991; Taylor et?al., 1996; Sanders et?al., 1993; Yuki et?al., 2014; Kollewe et?al., 2015); GQ1b among ALS is a very rare occurrence (Sawaya, 2019). A review of literature does not review any case where ALS is diagnosed in a patient who recovered from MFS with re-surging GQ1b levels as the common finding. 1.1. Case A 68-year-old Hispanic female with past medical history of hypertension, diabetes mellitus, familial hyperlipidemia, and asthma presented to our hospital complaining of dizziness, bilateral external ophthalmoplegia, diplopia, and lower limb ataxia with altered gait that had been present for three days. She had a nonspecific upper respiratory tract infection three weeks prior. She did not receive any vaccination in the past 6 months. There was no travel history or sick contact. On physical examination, she was alert and oriented to person, place, and date. No focal sensory or motor deficit was present. Deep tendon reflexes were found to be absent in all extremities and she had an unsteady gait without any lateralization. Rapid alternating movement was mildly impaired, though the patient did not have evidence of hand tremor, shuffling gait, or slurred speech. Her pupils were round and equal with sluggish pupillary light reflex. Complete blood count, comprehensive metabolic panel, thyroid function, liver function and cardiac markers were unremarkable. The urine toxicology for common substances, alcohol level, and serum anti-cholinesterase antibody test were negative. MRI of the brain was unremarkable. Cerebral spinal fluid analysis showed WBC 2/l, RBC 0/dl, glucose level of 63 mg/dl, and protein level of 33mg/dl with negative gram stain and cultures. Hepatitis B surface antigen, HIV, and RPR were bad. Serum ganglioside antibodies IKBKB against GD1b, GM1, and GQ1b were sent to ARUP Laboratories. The individuals GD1b antibody was 146 and GQ1b DDR-TRK-1 antibody was 478 (normal range 0C50). The rest of the ganglioside antibody panel was bad. Electrodiagnostic studies were not performed. Her symptoms progressed over 1 week. At the worst, she had total ophthalmoplegia in all directions and severe DDR-TRK-1 bilateral ptosis. She experienced moderate dysarthria and dysphagia. She was able to protect her airway and no mechanical ventilation was needed. Arriving at the analysis of Miller Fisher Syndrome, intravenous immunoglobulin (octagram 5% 400mg/kg/day time x 5 days) was started. Due to the quick DDR-TRK-1 improvement of devastating symptoms, our patient was discharged and instructed to follow up with outpatient neurology. She was seen at a follow-up visit six weeks post discharge, with total resolution of initial symptoms and was completely asymptomatic. A repeat GQ1b antibody level was 135 on that outpatient check out, the rest of the antibody panel was bad. She experienced no deep tendon reflex on that check out. Interestingly, this individuals MFS was among a surge of instances explained by Liu et?al., in 2015 (Liu and Yang, 2020). During that year, there was a surge in both GBS and MFS instances with seasonal difference. MFS was particularly common DDR-TRK-1 accounting for 52% of the total cases. Fifteen weeks after the initial hospitalization, she returned to the hospital with progressive weakness, dysphagia, slurred conversation, and great difficulty standing up with progressively frequent falls. Physical examination showed normal mentation, severe dysphagia, and dysarthria. Attention exam was completely normal with no ophthalmoplegia. Motor exam was significant for generalized muscle mass atrophy with fasciculation most obviously seen in right upper extremity. There was a positive Hoffmans sign, hyperreflexia in knee jerks, sustained clonus in the ankle, as well as bilateral positive Babinski sign. There were no indications of bowel or bladder incontinence and her lower engine strength was 4-/5 throughout. Sensation exam was normal for light touch, proprioception, ice sensation, and noxious stimuli. Cervical spine MRI results.