Hemispherectomy is a neurosurgical procedure that is frequently performed in pediatric patients diagnosed with Rasmussen's encephalitis. Postoperative complications include immediate complications such as hydrocephalus and hemorrhage and behavioral complications such as language impairments and contralateral weakness. However, there are limited studies or case reports that address the potential endocrinopathies associated with this and other pediatric epileptic surgeries. This case report describes the endocrinopathies following an anatomical hemispherectomy procedure. A four-year-old African-American female had a right anatomical hemispherectomy for the treatment of Rasmussen's encephalitis in 2020. The postoperative course was immediately complicated by central diabetes insipidus which was stabilized with desmopressin. The patient's labs in 2021 were consistent with central precocious puberty with elevated luteinizing hormone (LH) and follicle-stimulating hormone (FSH). Additionally, the patient was found to have secondary adrenal insufficiency in which she failed a low-dose adrenocorticotropic hormone (ACTH) stimulation test. Oral hydrocortisone therapy was initiated for secondary adrenal insufficiency in addition to initiating leuprolide injections for central precocious puberty. Furthermore, at the age of seven years, the patient had her first menarche. This case report emphasizes the need for closer and long-term surveillance for endocrine issues in postepileptic surgical pediatric patients as well as a surveillance plan for the development of other potential endocrine abnormalities throughout the patient's life.
Rasmussen encephalitis (RE) is a rare and progressive form of chronic encephalitis that typically affects one hemisphere of the brain and primarily occurs in pediatric individuals. The current study aims to narratively review the literature about RE, including historical information, pathophysiology, and management of this condition. RE often occurs in individuals with normal development, and it is estimated that only a few new cases are identified each year in epilepsy centers.
Background: Rasmussen's encephalitis (RE) is a rare neurologic disorder characterized by progressive seizures and unilateral cerebral atrophy with onset during childhood and unknown etiology. When medical therapy appears refractory, surgical disconnection of the affected hemisphere is indicated. Quality of life after functional hemispherectomy is largely good, affected females may therefore pursue pregnancy.
Department of Epileptology, Krankenhaus Mara, Bethel Epilepsy Center, Medical School OWL, Bielefeld University, Maraweg 21, 33617, Bielefeld, Germany. Electronic address:
- The study surveys medical providers about their current practices regarding the diagnosis and treatment of Rasmussen syndrome (RS), a condition that results in brain atrophy and various neurological issues.
- Most providers conduct tests for autoimmune encephalitis, but there are significant differences in the use of genetic testing and biopsy across regions, especially between US and European doctors and those from elsewhere.
- The findings reveal varying opinions on treatment priorities, particularly between surgical options and immunotherapy, indicating a need for further research and the development of consensus guidelines in RS management.
The study assesses the effectiveness of cenobamate, a new antiseizure medication, in treating three patients with the severe epilepsy syndrome linked to Rasmussen's encephalitis, which had previously resisted over 10 treatments.
The results show that adding cenobamate to their existing treatment not only fully controlled their seizures but also eliminated episodes of focal motor status epilepticus and allowed for a reduction in overall medication.
The findings highlight cenobamate's potential as a strong treatment option for this rare epilepsy syndrome and suggest the need for further research into its mechanisms compared to traditional medications.
