It Takes Two to Make a Thing Go Wrong

Seventy-three-year-old diabetic male was a high-risk transfer from Alaska for respiratory decompensation in the setting of progressive bulbar and proximal weakness. He was diagnosed with COVID-19 two months prior and viral mononucleosis 1 month prior to presentation. While the patient had a fall 3 months prior to presentation, and decreased mobility at home, there was abrupt onset of progressive upper/lower extremity weakness, dysphagia, and difficulties managing secretions 2 weeks prior to presentation. Initial exam was notable for MRC 3-4/5 proximal upper/lower extremity weakness, areflexia, and negative inspiratory force of 224 to 230 cm H20. A subtle periorbital heliotrope rash was documented. Lumbar puncture demonstrated albumino-cytologic dissociation (protein 142 mg/dL, 6 WBCs) and CK remained elevated (1930 U/L) despite intravenous hydration. Outside electrodiagnostic testing demonstrated a sensorimotor axonal neuropathy with questionable myopathic features on needle electromyography. Given concern for an inflammatory neuropathy and concomitant inflammatory myopathy, intravenous immunoglobulin 2G/kg and IV methylprednisolone 1G/day over 5 days was started. He was transferred for further diagnostic workup and supportive care 6 days after presentation and required intubation within 24 hours of admission. Exam showed progressive proximal and distal weakness of the extremities and general areflexia/hyporeflexia. Repeat electromyography confirmed a severe sensorimotor axonal polyneuropathy without acquired demyelinating features and normal repetitive nerve stimulation. While the patient could no longer activate muscles voluntarily, proximal muscles had increased spontaneous activity with predominant myotonia. Neuroaxis imaging was notable only for enhancement of the lumbar nerve roots. Combined vastus lateralis muscle biopsy and serologic testing confirmed a second pathologic process contributing to the patient's weakness. This case highlights the cooccurrence of 2 distinct neuropathological entities, with potential relation to a prior viral infection, and the importance of ancillary testing to guide treatment for acute causes of neuromuscular respiratory failure.

A Pediatric Case of Sensory Predominant Guillain-Barré Syndrome Following COVID-19 Vaccination

Objective: Increased data about potential adverse events following COVID-19 vaccination will contribute to a better-informed evaluation of the safety of the vaccines. Here, we describe a case of sensory predominant Guillain-Barré Syndrome (GBS) following administration of PfizerBioNTech vaccine in a 16-year-old female. Background: Over 3.58 billion people worldwide have received at least 1 dose of the COVID-19 vaccine. The concerns for GBS first arose with ChAdOx1 nCoV-19 (Oxford/AstraZeneca). More than 200 cases associated with the vaccine have been reported. Recently, Janssen/Johnson&Johnson vaccine was also reported to have a small but statistically significant increase in risk for GBS with an estimated absolute rate increase of 6.36 per 100,000 person-years in adults. Thus far, no studies have shown an association between Pfizer-BioNTech vaccine and GBS. Design/Methods: N/A Results: A previously healthy and athletic 16-year-old female presented to the emergency department with 3 weeks of ascending numbness and paresthesia of her bilateral lower and upper extremities. She received her second dose of Pfizer-BioNTech COVID-19 vaccine 2 days prior to symptom onset. On neurological examination, she had intact strength, absent reflexes, decreased sensation to pinprick, diminished vibratory sensation, and mild ataxia on toe and tandem walking. MRI study showed mild thickening and enhancement of the anterior and posterior spinal nerve roots of the cauda equina, consistent with GBS. With lumbar puncture, albuminocytologic dissociation was seen in CSF. Conclusions: Our patient presented with clinical signs, imaging, and lab findings most consistent with the diagnosis of GBS. Idiopathic or asymptomatic infectious causes of GBS cannot be completely ruled out;however, with the recent vaccination and absence of any other clinical signs or lab findings, the vaccine is most likely to be the trigger for our patient. To date, this is the first case reported of GBS in a pediatric patient after receiving the Pfizer-BioNTech vaccine.

Different Guillain-Barré Syndrome Variants Associated with COVID-19: Report of 4 Clinical Cases

Since the first case has emerged, different neurological complications associated with Coronavirus disease-2019 (COVID-19) have been reported all over the world. The association between coronavirus and Guillain-Barré syndrome (GBS) has also been reported before. Unfortunately, there is no certain mechanism about this association yet. Molecular mimicry is one of the first hypotheses to express the undesirable autoimmunity in GBS. According to this;the antibodies produced against virus may target peripheral nerves or spinal nerve roots. The entity of direct viral neurotoxicity has also been discussed. Here, we presented four variants of GBS associated with coronavirus that we followed up in our clinic between September-December 2020. While the first patient had demyelinating sensorimotor neuropathy concurrent with COVID-19, the other ones were postinfectious. The second patient had motor axonal neuropathy and the third one had sensorimotor axonal neuropathy. There was Miller-Fisher syndrome and GBS overlap in the fourth patient.