summer 2019 | volume 33 | Issue 2 A journal of Dayton Children’s Hospital acute flaccid myelitis: a case study in the pediatric intensive care unit by Kristin Mikolajewski, MSN, CPNP-AC/PC 1 learning objectives The patient’s admission exam was significant CSF Analysis: Following the completion of this article, for a left-sided facial Colorless and clear, the reader should be able to: droop, asymmetric Glucose 61 mg/dL, 1. Discuss diagnostic criteria for acute smile and inability to Protein 52 mg/dL, flaccid myelitis. completely close left WBC 18 cell/mm3 2. Review management and treatment eyelid. Pupillary reflexes (HIGH), RBC 28 options for acute flaccid myelitis. were normal. He was cell/mm3 (HIGH), 3. List complications associated with unable to shrug his Lymphocyte 76% acute flaccid myelitis. right shoulder or lift (HIGH), Eosinophil his right arm against 2%, Monocyte/ gravity. He had right Macrophage 22% upper extremity hy- CSF culture negative A previously healthy 13-year-old poreflexia and proximal (including fungal) unimmunized male presented to the weakness (shoulder emergency department with a one-day strength 2/5, elbow CSF Enterovirus strength 4/5, hand PCR negative history of right shoulder weakness and grip normal). All other CSF HSV PCR negative neck stiffness. Symptoms started extremities had normal approximately one week prior with fever strength and tone. CSF mycoplasma (103°F), sore throat, nasal congestion and He had no sensory pneumoniae IgM deficits. He was able and IgG positive dizziness. He was initially evaluated by his to ambulate and sit CSF Borrelia primary care physician, diagnosed with upright but required burgdorferi antibody head support. He was sinusitis and prescribed a course of negative alert and oriented with amoxicillin. Two days later, he developed normal speech. CSF oligoclonal left-sided facial droop and difficulty bands negative Initial lab workup swallowing. He was taken to the local ED was notable for a CSF Aquaporin-4 where he was diagnosed with Bell’s palsy positive rhinovirus/ antibody negative and prescribed a course of oral steroids. enterovirus PCR CSF arbovirus (polymerase chain The following day he developed right panel negative reaction) from shoulder weakness as well as neck stiffness. respiratory secretions, ANA negative He returned to the local ED where he a lumbar puncture Double stranded DNA showing cerebrospinal underwent MRI and CT scan of the head antibody negative fluid (CSF) pleocytosis and cervical spine which were both normal. (WBC 18/mm3) and a HSV PCR blood Due to the severity and rapid progression of positive mycoplasma negative symptoms, he was transported to the local pneumoniae CSF Stool viral culture antibody screen. children’s hospital for further evaluation. negative His CBC and BMP Additional history was noncontributory. were normal. Respiratory PCR No recent travel. No insect bites. No panel POSITIVE for vomiting. No diarrhea. No rash. No bowel Rhinovirus/enterovirus or bladder dysfunction. No known ill *repeat 48 hours later was negative exposures. Home medications included Blood culture negative ibuprofen for fever as well as the prednisone and amoxicillin as Urine culture negative previously prescribed. table 1. Laboratory data 2 with early identification from the blood. It is of respiratory failure. frequently used in the treatment of neurologic Despite 72 hours of disorders with aggressive steroid and autoimmune etiology.) antibiotic therapy, the patient’s condition The patient remained worsened with weak- hospitalized for 13 days. ness spreading to his He received a total of left upper extremity. five days of high dose He underwent repeat IV steroids, five plasma MRI which now showed exchange transfusions concern for cervical and 14 days of myelitis with a spinal doxycycline for the cord lesion extending treatment of from the level of the mycoplasma brainstem down to the pneumoniae. The level of C7 (figure 1). ceftriaxone and acyclovir were A team of physicians discontinued after 48 from neurology, hours with negative infectious disease, CSF, blood and urine immunology, pediatric cultures. His respiratory critical care, neurosur- status remained stable, gery, pediatric surgery, requiring only a brief physical medicine and period of supplemental rehabilitation, and oxygen via nasal nephrology reviewed cannula. At time of the case. The team discharge, the patient concluded that this figure 1. MRI image demonstrating increased was able to support patient’s symptomatol- signal within the spine extending superiorly his own head, ogy and MRI findings into the brainstem starting at the level of the ambulate independent- were most consistent middle cerebellar peduncle and extending ly and tolerate a regular with acute flaccid downward into the medulla and cervical spinal diet by mouth. He had myelitis (AFM), an cord to the level of C7. minimal improvement acute neurologic in his extremity disorder characterized weakness or facial by unilateral Due to high risk for A nasogastric feeding palsy. On six-month flaccid weakness. respiratory failure, the tube was placed for follow-up, the patient While there are no patient was admitted nutrition due to cranial had completed evidenced-based to the pediatric nerve dysfunction and extensive rehabilitation treatment guidelines intensive care unit. aspiration risk. Physical therapy consisting of for AFM, there are case He was started on therapy, occupational two hours a day, five studies documenting empiric treatment therapy and speech days a week. He had improved outcomes for meningitis and therapy were consulted resolution of left upper with steroids and transverse myelitis, for early rehabilitation extremity weakness. plasma exchange. which included treatment. NIFs His right shoulder At this point, the methylprednisolone (negative inspiratory weakness and left 30 mg/kg/day, force) and exhaled CO medical team decided 2 facial palsy were to proceed with ceftriaxone 100 mg/kg/ via nasal cannula were present but improved. plasma exchange. day and acyclovir 30 monitored to assist The probability of full (Plasma exchange, or mg/kg/day. recovery is unknown, plasmapheresis, is a but he continues with procedure in which therapy in hopes inflammatory of continued mediators are filtered improvements. 3 defining acute transverse Guillain-Barre acute disseminated spinal cord characteristics flaccid myelitis myelitis syndrome encephalomyelitis infarct Encephalopathy not present not present not present present not present Cranial nerve may or may not present may or may may or may not present dysfunction not be present not be present not be present Muscle tone decreased, initially decreased, variable decreased often unilateral, decreased, ascending in UE > LE increased in nature, later stages, often often bilateral, symmetric, LE > UE LE > UE Sensory typically not present present may or may present deficits present (clearly defined not be present sensory level) Reflexes decreased acutely decreased variable decreased decreased, chronically increased CSF Pleocytosis present present not present present not present table 2. Differential diagnosis of acute flaccid myelitis LE = lower extremities UE = upper extremities discussion high dose steroids and background hyporeflexia plasma exchange. Both or arefelxia and The definitive AFM is a rare, polio-like of these therapies have hypotonia. Cranial diagnosis for this case illness that is character- been associated with nerve dysfunction was challenging as the ized by acute onset of positive outcomes in occurs in 30 percent of patient’s symptom- flaccid weakness of one ATM and AFM. cases2. Sensory deficits atology did not meet or more extremities Additionally, while the are rare, but if present the classic criteria for a with specific MRI etiology of his illness are usually mild and single disease process. findings of lesions was never defined, transient. MRI findings This patient exhibited within the anterior the labs suggested an are most common in cranial nerve involve- horns of the gray infectious causality the cervical spine. The ment, which is less matter of the spinal related to enterovirus gray matter lesions likely with AFM. cord1. The illness or mycoplasma are initially diffuse However, the unilateral- typically occurs in pneumoniae. Both but become more ity of his weakness children (median age 6 of these pathogens localized to the anterior and the gray matter years) and affects more have documented horn cells over days to findings on MRI were males than females. associations with AFM weeks. CSF pleocytosis more characteristic of The onset is usually and ATM. The possi- is present in 80 AFM. One could argue rapid (within hours to bility of poliomyelitis percent of cases3. Many that this case meets days) and asymmetric. was discussed given his patients experience a the criteria for an The upper extremities unimmunized status, prodrome of respira- atypical presentation are more predominant- but was later ruled out tory or gastrointestinal of either acute trans- ly affected than the with negative stool viral symptoms, which verse myelitis (ATM) or lower, and the proxi- panel. The lack of data may include fever, AFM (table 2). In fact, mal muscle groups are surrounding the treat- approximately five some experts argue more predominantly ment of AFM made this days prior to onset of that AFM is a subset affected than the case challenging, and weakness2,4. Prognosis of ATM. Nonetheless, distal2. Patients exhibit highlights the need for is not well understood. this patient showed