Objective:

To describe an incidentally discovered, asymptomatic case of Central Pontine Myelinolysis (CPM) in a child evaluated for fever of unknown origin, subsequently diagnosed with Hodgkin's Lymphoma.

Background:

Central pontine myelinolysis is a rare demyelinating brainstem disorder, typically secondary to rapid correction of hyponatremia. This classically causes encephalopathy, seizures, delayed spastic quadriplegia, bulbar impairment, or locked-in syndrome. Asymptomatic presentations are exceptionally rare, especially in pediatric populations, and cases unassociated with osmotic changes are sparsely reported. Preventive management remains key.

Design/Methods:

A single patient case report.

Results:

A previously healthy ten-year-old male presented with six months of fever, cough, fatigue, weight loss, and mid-back pain.  Tuberculosis testing was negative. Inflammatory markers were elevated (ESR 69 mm/hr; CRP 9.05 mg/dL). CT chest/abdomen/pelvis showed pulmonary lesions, bulky lymphadenopathy, and splenomegaly. MRI spine demonstrated multiple enhancing vertebral and pelvic lesions. Subsequently, MRI brain unexpectedly revealed a well-circumscribed central pontine lesion with T2/FLAIR hyperintensity and restricted diffusion without enhancement consistent with CPM. While CPM is commonly associated with sodium derangement and liver disease, this patient’s electrolytes and liver function tests were normal. Serial neurologic examinations remained normal. Initial biopsy of the pelvic mass and bone marrow aspiration were nondiagnostic. Surgical biopsy later confirmed Stage IVb Nodular Sclerosing Hodgkin’s Lymphoma. Patient showed excellent response to chemotherapy per later PET scan with resolution of the pontine lesion confirmed via MRI. He remained neurologically asymptomatic throughout his disease course.

Conclusions:

This case highlights an exceptionally rare presentation of asymptomatic CPM in a child with Hodgkin’s Lymphoma without any noted metabolic derangements. In more than one hundred reported pediatric cases of CPM, none have been asymptomatic or associated with malignancy. Recognition of incidentally-occurring CPM outside its classical context is critical, and neurologic examination and follow-up imaging are warranted to monitor either evolution or resolution of pontine lesions.