TOP TO BOTTOM: ATYPICAL GUILLAIN-BARRE PRESENTATION IN A PATIENT WITH CEREBRAL PALSY Ross Dies, Farhan Mohiuddin, Seth Vignes; Louisiana State University, New Orleans, LA.
Introduction: Guillain-Barre Syndrome (GBS) is an acute immune-mediated polyneuropathy that typically presents with ascending paralysis, paresthesia, cranial nerve involvement, and limb pain. Respiratory muscle involvement is common and can quickly progress to respiratory failure. The diagnosis is primarily clinical, and atypical presentations may lead to missed diagnoses. Identification may be especially difficult in those with baseline neurological deficits. Case: A 53-year-old woman with asthma and cerebral palsy presented for right leg pain in the area of a chronic wound, along with dysphagia and dysarthria. At baseline, she was bedbound with bilateral leg pain, spasms, and decreased strength. She was found to have thrush and underwent empiric treatment in addition for cellulitis. Within a few days of admission, her symptoms had worsened to include choking during meals and drooling, along with new weakness, areflexia, and paresthesia in the bilateral upper extremities (BUE). Consultation with specialists at the Louisiana Department of Health and the Centers for Disease Control and Prevention indicated low likelihood of botulism as the cause. The decision was made to obtain cerebrospinal fluid (CSF) studies and begin empiric intravenous immunoglobulin (IVIG). CSF studies
revealed albuminocytologic dissociation without evidence of infectious process, consistent with GBS. This information, combined with rapid declines in negative inspiratory force (NIF) testing, prompted transfer to the intensive care unit and eventual intubation with mechanical ventilation. Transient episodes of tachycardia and hypothermia occurred, representing possible autonomic dysfunction. She was able to be extubated sometime later, but her dysphonia and BUE weakness were much slower to improve. After a three-week stay, she required an additional month in an inpatient rehabilitation facility, with residual neuropathy and eventual partial improvements in muscle tone and strength. Discussion: This case represents the variability in presentations of GBS, particularly in individuals with already present neurological disease. Although not progressing as suspected, this patient’s symptoms somewhat suggested GBS. Physicians should have a low threshold to consult neurology and obtain CSF studies if the diagnosis is in question. Delays in diagnosis and treatment have been associated with higher rates of intubation, along with increased time to recovery or even permanence of neurologic sequelae.
TOXIC SHOCK SYNDROME IN A CASE OF GROUP A STREPTOCOCCAL PNEUMONIA Ross Dies, Farhan Mohiuddin, Justin Ellerman, Megan Rojas, Shelby MacRae; Louisiana State University, New Orleans, LA.
Introduction: Toxic shock syndrome (TSS) is an acute toxin-mediated condition caused by certain strains of Staphylococcus aureus and Streptococcus pyogenes, or group A strep (GAS). Toxins produced by these strains, known as superantigens, promote massive cytokine release, which rapidly results in hypotension and end-organ damage. GAS- mediated TSS is most often secondary to soft tissue infection, pharyngitis, and bacteremia, but rarely may stem from a focal pneumonia. Associated mortality rates can be as high as 60%, and even higher in cases of missed or delayed diagnoses.
Case: A 54-year-old male with an extensive past medical history presented with shortness of breath, acutely worsening over several days and proceeded by pharyngitis and cough. Notable physical exam findings included a red macular rash on the abdomen and conjunctival hyperemia. Due to respiratory distress, he eventually required intubation. Subsequent chest imaging revealed left lower lobe pneumonia. Broad-spectrum antibiotics and insulin were initiated, and he was admitted to the intensive care unit for treatment of presumed sepsis and diabetic ketoacidosis.
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