RESUMEN
Background: Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is a relatively new disease entity in the field of demyelinating disorders. Its first diagnostic criteria have recently been published. Objectives: We evaluated the positive predictive value (PPV) for MOG-IgG testing and report the clinical and radiologic features with respect to the recently published criteria. Methods: A retrospective study was conducted at three centers in Dallas, Texas. Patients with positive MOG-IgG testing on cell-based assays at any time were included. Positive cases were reviewed by at least two neuroimmunologists for fulfillment of the criteria. Results: We included 235 patients. The PPV of seropositivity at any time was 78.3% overall, 52.6% for low titer, and 90.1% for high titer. Children had a higher PPV than adults (93.9% versus 67.2%). Positive predictive value was 6.3% in those without a core clinical demyelinating attack. Children more often have the typical imaging features of MOGAD in optic neuritis than adults. Conclusions: We report a PPV of 78.3% for MOG-IgG testing using the 2023 MOGAD diagnostic criteria. Children had higher PPV and frequency of supporting imaging features. Careful consideration is necessary when assigning patients with no core demyelinating event and low titers a MOGAD diagnosis.
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Recurrent painful ophthalmoplegic neuropathy is a form of cranial neuralgia and rare source of pediatric headache. We present 2 children who presented with headaches accompanied by visual symptoms including eye pain, blurry vision, and diplopia. MRI in both patients demonstrated enhancement of the cisternal segment of the oculomotor nerve in the affected side, correlating with the observed symptoms.
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Menkes disease is a rare X-linked recessive disorder caused by impaired copper absorption and transport. Presented here is a case of a 12-week-old male who presented with hypotonia and multiple metaphyseal fractures. Further imaging and workup revealed a diagnosis of Menkes disease. While nonaccidental trauma is a much more common cause of metaphyseal bone fractures, encephalopathy, and subdural hematomas in infants, Menkes syndrome should be considered in the setting of corroborating signs and symptoms, as early diagnosis and treatment can delay progression of the disease.
RESUMEN
Neurotoxicity is a rare side effect of metronidazole therapy. Shown here are findings of metronidazole toxicity in a patient, who received chronic metronidazole as prophylaxis for pseudomembranous colitis following bowel resection as an infant. Findings depicted include increased T2 signal in the dentate nuclei and brainstem. Discontinuing the medication resulted in reversal of the findings.