On the morning of July 6, Children's Hospital 2, Ho Chi Minh City, shared about the emergency treatment and successful treatment of two children with a rare epilepsy syndrome, hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome. These are two rare cases of HHE syndrome in the world and were successfully treated by doctors at Children's Hospital 2.
According to Specialist Doctor 2 Do Chau Viet, Head of the Infectious Intensive Care Department, Children's Hospital 2, when receiving two pediatric patients, both female: NHX (born in 2020) and LTN (18 months old), living in Ho Chi Minh City with a condition of prolonged hemiplegia, causing hemiplegia on the same side of the seizure and cerebral atrophy on the opposite hemisphere. After a series of paraclinical tests, a consultation of interdisciplinary doctors determined that both had hemiplegia-hemiplegia epilepsy syndrome.
HHE syndrome is a rare disease, first discovered in 1960 and recorded in medical literature as very rare. In the acute phase, patients experience many uncontrollable seizures, damage and edema of one cerebral hemisphere. If not well controlled, patients may have lifelong hemiplegia, brain sequelae and vegetative life, and may even have brain herniation leading to death. HHE is diagnosed based on characteristic brain imaging on MRI. In the acute phase, there are signs of edematous lesions in the cerebral hemisphere, followed by cerebral atrophy that does not correlate with any vascular area.
NHX child patient with rare epilepsy syndrome recovered after treatment. |
When admitted to the hospital, LTN had a high fever of 39 degrees Celsius with prolonged generalized convulsions, with generalized convulsions alternating with convulsions on the right side of the body. The patient was intubated by doctors and transferred to the Infectious Intensive Care Unit. Brain MRI results showed that the child had diffuse limited lesions in the left cerebral hemisphere while the right cerebral cortex was normal. Because the child's other test results were negative for encephalitis agents such as Herpes simplex, Japanese encephalitis, and no bacteria were found on examination and culture, the child was treated with high doses of steroids, breathing assistance, and anti-cerebral edema. After 3 days of active treatment with anti-epileptic drugs, combined with physical therapy, LTN has now fully recovered in terms of perception, muscle strength, and muscle tone throughout the body.
For patient NHX, who was hospitalized with multiple seizures with generalized convulsions alternating with convulsions of the mouth and right hand, coma... " The MRI results of baby X's brain also showed damage with cerebral edema almost in the entire left hemisphere, shifting the midline to the right, and the right hemisphere was compressed. The doctors of the department quickly consulted and used high-dose steroids 30mg/kg/day for 5 days, and at the same time used 1 gram/kg/day of Globulin antibody intravenously for 2 days. After 10 days of active treatment, the patient recovered well and was able to be weaned off the ventilator" - Specialist Doctor 2 Do Chau Viet shared.
News and photos: NGUYEN TAM
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