This report describes five patients who, following a neurologically complicated primary Epstein-Barr virus infection, developed progressive or relapsing neurologic deficits. The sequelae in four patients followed 4 to 12 years led to the diagnosis of multiple sclerosis (MS). The fifth patient presented with acute disseminated sclerosis and exhibits diffuse neurologic deficits that have persisted for 2 years. We suggest that the diagnosis of an unexplained acute neurologic or psychiatric syndrome should raise the question of a primary EBV etiology. A precisely timed serologic and hematologic study of the blood is imperative to capture the essential evidence. The data presented represent a clinical association between a neurologically complicated primary EBV infection and both chronic and acute demyelinating disease. The evidence does not justify a conclusion that EBV virus causes MS.
A 17-year-old man with the Kleine-Levin syndrome died unexpectedly of cardiopulmonary arrest during a period of autonomic instability that followed an episode of megaphagia. At autopsy, the only pertinent finding was mild depigmentation of the locus ceruleus and substantia nigra. Premortem CSF levels of 5-hydroxytryptamine (5-HT) and 5-hydroxyindoleacetic acid (5-HIAA) levels were elevated. These findings indicate that many symptoms of the Kleine-Levin syndrome are a result of a neurotransmitter imbalance in the serotonergic pathway of the brainstem.
Seizure reduction was the same in patients younger than 12 years and 12 years or older and in patients with shorter and longer histories of refractory epilepsy. Adverse effects were few in this population, particularly in those younger than 12 years. Vagal nerve stimulation appears to be a relatively safe and potentially effective treatment for children with severely intractable epilepsy.
Computed tomography (CT) scans provide three-dimensional information about intracranial structures, which can be used to place stereotactically guided radiofrequency (RF) lesions and destroy a targeted volume of tissue. This technique was used for lesioning of the corpus callosum (CC) or the amygdala-hippocampus complex (AHC) in 9 patients with intractable seizures. The procedures were monitored by intraoperative CT scans. Lesions were made in the AHC in 7 patients and the CC in 2 patients. In addition, multiple subpial transection (MST) was performed in 6 patients. The longest follow-up is 29 months with a median of 19 months. Five patients (56%) are free of seizures, 3 patients (33%) have greater than 90% reduction in seizure activity and 1 patient (11%) has greater than 50 % but at most 90 % reduction in seizure activity. There were no complications except for temporary hemiparesis following MST in 1 patient. The results suggest that stereotactic volumetric RF lesioning of the AHC and the CC may be safe and effective in controlling intractable seizures.
Vagus nerve stimulator implantation has a low incidence of serious complications. Quality of life seems to be improved for most patients. Modifications to the surgical procedure must be considered when performing the implantation on a young patient.
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