Virtual Conference
Pharma Conference 2022

Nailya F. Ahmetgaleeva

Children's Center of Psychoneurology and Epileptology of the Republican Children's Clinical Hospital, Russian Federation

Title: Central pontine myelinolysis in a child: Case report


Central pontine myelinolysis (CPM) is an acute condition accompanied by symmetrical demyelination of the brain pons (Sheveleva E.M. et al., 2019). CPM is the variant of osmotic demyelination syndrome (ODS). The incidence of central pontine myelinolysis is 40-56% among all patients with ODS. Pathology is rare in the population. Hereditary susceptibility and statistically significant gender differences in incidence were not found.

Cases of the disease in children are casuistic (Damulin I. et al., 2018). Central pontine myelinolysis was first described in 1959 as a disease of "malnutrition" and "alcoholics"; The term "myelinolysis" is used to distinguish the condition from inflammatory demyelination of nerve fibers. Electrolyte imbalance dew to rapid increase osmolarity in blood underlying the pathogenesis of the CPM (Duduk S.L. et al., 2012). Oligodendrocytes are most susceptible to osmotic damage, hereby axons remain intact, which explains the reversibility of the pathological process. The macroscopic presentation of the pons often shows "batwings" that may extend into the midbrain. Histological examination is characterized by loss of myelin of nerve fibers (Savin A.A et al., 2017).

The clinical picture in the initial period corresponds to the manifestations of hyponatremia: depression of consciousness, repeated vomiting, convulsive seizures. Later, signs of proper demyelination of nerve fibers appear - increased muscle tone, hyperreflexia, pathological reflexes due to damage to the corticospinal tracts Magnetic resonance imaging (MRI) is the main method of instrumental confirmation of the diagnosis of CMP. In this case, the most characteristic changes are detected in the T2-weighted mode: with CPM, a zone of a uniform hyperintense signal extends to the medial part of the base of the pons of the brain. On T1-weighted images, a moderately hypointense signal from lesions is most often noted. MRI in diffusion-weighted mode (DWI) is characterized by the presence of a hyperintense signal (Forster A. et al., 2013).

Central pontine myelinolysis is not a specific syndrome. Liver damage, endocrine and metabolic disorders are prerequisites for the occurrence of this condition. In children, osmotic demyelinating syndrome is extremely rare, apparently due to the absence of predisposing factors and accompanying diseases. As a rule, the changes are reversible because of nervous tissue plasticity. Every new case of CPM in children has an undeniable interest and high scientific and practical significance due to its low incidence.


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