Isolated extrapontine myelinolysis with neuropsychiatric manifestation: A case report from Nepal

A referred case of a 34-year-old woman was brought to the emergency room with features of mutism and whole-body rigidity following rapid correction of hyponatremia. On presentation, she was ill looking with tremors on her both hands and legs. She was unresponsive to command and developed involuntary outbursts of laughter and crying. Based on clinical features and neuroimaging, diagnosis of isolated extrapontine myelinolysis was made. She was treated with quetiapine, vitamin B1 and B12 supplements, trihexyphenidyl, levodopa-carbidopa and physiotherapy of limbs. Due to lack of clinical trials for adequate diagnosis and management of extrapontine myelinolysis, this case report highlights the importance of extrapontine myelinolysis with neuropsychiatric manifestation in research world.

A highly unusual case of osmotic demyelination syndrome and extrapontine myelinolysis in a 3-month-old infant with Bartter syndrome

Bartter syndrome (BS) is a rare autosomal recessive renal tubular disorder characterized by acute electrolyte imbalance, and similarly, osmotic demyelination syndrome (ODS) is a rather rare complication occurring during electrolyte imbalance. The pathological features of ODS include central pontine myelinolysis and extrapontine myelinolysis (EPM), which consist of severe damage to the myelin sheath of neurons. ODS is very rare in children. We describe a case of a 3-month-old infant with ODS and EPM associated with undiagnosed BS. ODS developed because of a sudden change in electrolyte levels and osmolality caused by acute dehydration during a gastrointestinal infection episode. Undiagnosed, untreated, and non-balanced BS was the cause of the neurological complication. Our patient represents the first case of ODS in BS, the ninth case of ODS in an infant less than one year old, and the third case of isolated EPM in such a young patient. This case report reminds us that in rare diseases, young patients tend to have genetic components.

Central pontine myelinolysis and extrapontine myelinolysis: literature review and case report

Central pontine myelinolysis (CPM) is a concentrated, symmetric, non-inflammatory demyelination within the central basis pontis. In 10 % of patients with СРМ, demyelination also occurs in extrapontine regions: the mid brain, thalamus, basal nuclei, and cerebellum. Demyelination occurs in regions of compact interdigitation of white and gray matter as a result of cellular edema, which is caused by fluctuating osmotic forces, results in compression of fiber tracts. The most frequent cause of abrupt change in osmotic pressure is the rapid sodium correction of prolonged hyponatremia. In case that we report, there is a combination of central pontine and extrapontine myelinolysis, which occurs in 71-year-old woman owing to rapid sodium correction of prolonged hyponatremia which by turn developed due to salt-free diet, improper antihypertensive therapy and overheating. The diagnosis was confirmed by magnetic resonance imaging (MRI). The treatment led to significant decrease of neurological deficit.