Paroxysmal sympathetic hyperactivity following status epilepticus in a 22-year-old with Juvenile Neuronal Ceroid Lipofuscinosis: A case report.

The Neuronal Ceroid Lipofuscinosis (NCL) refers to a group of rare neurolipidosis disorders characterized by progressive blindness, deterioration of speech and motor function, cognitive decline, behavior problems, seizures, and premature death. We report a case of a 22-year-old man with CLN3 variant, homozygous NCL (aka Juvenile Neuronal Ceroid Lipofuscinosis) complicated by epilepsy who presented with episodes of recurrent seizure-like activity following status epilepticus, but now without electrographic correlate. Episodes were accompanied by tachycardia, diaphoresis, hypertension, and a fearful facial expression likely representing paroxysmal sympathetic hyperactivity (PSH), and improved with administration of propranolol.

Overlap between dissociation and other psychological characteristics in patients with psychogenic nonepileptic seizures.

Patients with psychogenic nonepileptic seizures (PNES) often report symptoms of dissociation. However, it is unclear how these symptoms relate to psychotherapeutic treatment, for example, with cognitive-behavioral therapy (CBT). Here, we investigated the degree of overlap between symptoms of dissociation and other psychiatric features that are more traditional targets for CBT.