Central Pontine and Extrapontine Myelinolysis in an Infant Associated With the Treatment of Craniopharyngioma -Case Report-

Abstract

A 3-year-old girl presented with osmotic demyelination syndrome after undergoing uneventful neuroendoscopic cystostomy for a growing cystic suprasellar craniopharyngioma following microscopic subtotal resection 1 year previously. Endocrinopathy had well been controlled by hormone replacement therapy and administration of 1-amino-8-d-arginine-vasopressin with serum sodium concentration within the normal range. She presented generalized seizure and fever on postoperative day 7, with hyponatremia beginning on postoperative day 4 and deteriorating despite frequent correction. The serum sodium concentration began to fluctuate on the same day, in the range 111-164 mEq/l, which lasted for 2 weeks, refractory for intense management. Her body temperature also fluctuated between hypoand hyperthermia not correlated with serum inflammatory markers. Her conscious disturbance progressively deteriorated with spastic paraparesis. T 2 -weighted magnetic resonance (MR) imaging taken on postoperative day 19 revealed hyperintense areas in the pons, external capsule, bilateral thalami, and basal nuclei, which had not been recognized before, suggesting osmotic demyelination syndrome causing central pontine and extrapontine myelinolysis. MR imaging taken on postoperative days 230 and 360 showed some diminished lesions but others persisted and resulted in a cavity. The patient's depressed conscious level did not improve. Suprasellar craniopharyngioma with long-standing hypothalamic dysfunction may be associated with severe osmotic demyelination syndrome even after less invasive surgery, so serum sodium derangement after surgery should be promptly corrected even if only subtle signs are present.

Achados de fala, deglutição e qualidade de vida na mielinólise extrapontina: relato de caso de uma adolescente com germinoma do sistema nervoso central

“…Some authors describe full neurological recovery 16 , while others partially recovery presenting neurological sequelae 5,6,13,14 , as in the case reported in this present study, which dysarthria remained in the patient.…”

“…The CPM and EPM are reunited under the title of osmotic demyelination syndrome and are frequently related to rapid hyponatremia correction [2][3][4] . The CPM is the most common disorder being followed by the EPM in 10-30% of the cases 13 . The EPM can be isolatedwithout involving the central pontis region -and is rare in pediatrics population 13,14 .…”

“…The CPM is the most common disorder being followed by the EPM in 10-30% of the cases 13 . The EPM can be isolatedwithout involving the central pontis region -and is rare in pediatrics population 13,14 .…”

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

“…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]. …”

“…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.…”