Central pontine myelinolysis in a 7-year-old boy

Kepes, John J.; Reece, Carol Ann; Oxley, Dwight K.

doi:10.1136/jnnp.28.1.39

Cited by 25 publications

(15 citation statements)

References 8 publications

(7 reference statements)

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“…Of these, and counting our own, 10 developed osmotic myelinolysis at some time after a neurosurgical procedure [24,25,26,27,28,29,30,31]. Ages ranged from 3 to 18 years, with 4 children younger than 10 years, and six 10 years or older (table 1).…”

Section: Discussionmentioning

confidence: 99%

“…In 3 cases, CPM was diagnosed, 5 had EPM and 2 had both. In 7 of these patients, neurological symptoms suggestive of myelinolysis started within 3 weeks of surgery [24,25,26,27,28,29,31], often within days. However, in 2 cases, our own and the patient of Niehaus et al [30], neurological symptoms started several years later, in patients receiving long-term treatment for postoperative panhypopituitarism.…”

Section: Discussionmentioning

confidence: 99%

“…However, in 2 cases, our own and the patient of Niehaus et al [30], neurological symptoms started several years later, in patients receiving long-term treatment for postoperative panhypopituitarism. Six of the initial surgeries had been to resect a craniopharyngioma [25,26,27,28,29,31], with 1 each to resect a suprasellar germinoma [24], a suprasellar mixed germ cell tumor [24], a neuroblastoma [30] and a nasofrontal encephalocele (current case). The patient with neuroblastoma had also received brain irradiation [30], and the patient with a germinoma had received chemotherapy [14] prior to the onset of CPM or EPM.…”

Section: Discussionmentioning

confidence: 99%

“…One of the worst outcomes is the ‘locked-in syndrome’ [1,12,13,14], but other severe neurological deficits, coma and death have been described. One of the most common surgical scenarios in which CPM and EPM have been reported has been after liver transplantation [15,16,17,18,19,20,21,22,23], but it has also been described in patients who develop diabetes insipidus or panhypopituitarism following intracranial surgery [24,25,26,27,28,29,30,31]. …”

Section: Introductionmentioning

confidence: 99%

“…We describe a case of fatal ODS that developed suddenly in a 13-year-old boy who previously had been successfully managed with corticosteroids, deamino arginine vasopressin (DDAVP) and thyroid hormone for postoperative panhypopituitarism for over a decade following the resection of a nasofrontal encephalocele in infancy. The literature on similar cases, including both neurosurgery patients [24,25,26,27,28,29,30,31] and patients on treatment for hypopituitarism [33,34,35,36], is reviewed. This case serves to emphasize the importance of sudden osmolar shifts in the genesis of ODS.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Osmotic Myelinolysis with Malignant Cerebellar Edema Occurring after DDAVP-Induced Hyponatremia in a Child

Ranger¹,

Szymczak²,

Levin³

et al. 2010

Pediatr Neurosurg

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Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are dire neurological disorders, characterized by severe damage to the myelin sheath of neurons, which typically result from rapid correction or overcorrection of systemic hyponatremia. For many years, both conditions have been considered universally fatal, though survivors have been reported more recently. Pediatric cases are rare. We present a 13-year-old boy with panhypopituitarism secondary to repair of a nasofrontal encephalocele in infancy, managed on long-term corticosteroid, deamino arginine vasopressin and thyroid hormone. He presented with severe hyponatremia (116 mEq/l), which during correction rapidly and unexpectedly increased to 176 mEq/l, resulting in profoundly impaired consciousness. Brain imaging revealed multiple bilateral changes in the basal ganglia, thalamus, pons and cerebral white matter, consistent with both CPM and EPM. Malignant cerebellar edema necessitated emergent suboccipital craniectomy, with subsequent improvement in level of consciousness and imaging postoperatively. However, he succumbed to acute cardiorespiratory arrest 8 weeks later. Nine similar cases from the literature are reviewed.

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