Background/Purpose: Neuromyelitis optica (NMO) is a rare inflammatory disorder of the central nervous system (CNS), characterized by recurrent optic neuritis (ON) and transverse myelitis (TM). Pediatric patients often show other affected areas in the CNS, such as postrema, diencephalon, or brainstem. In adult patients, up to 75% present with antibodies against aquaporin-4 (AQP4-ab) in the serum. These findings resulted in a revision of previous diagnostic criteria and led to the new unifying term NMO spectrum disorders (NMOSD). In AQP4-ab-naive patients, antibodies against myelin oligodendrocyte glycoprotein (MOG) could be found. These have fewer and less severe episodes and convalesce faster. For patients with AQP4-ab-positive NMOSD, a subsequent immunosuppression is recommended; in MOG-ab-positive cases, only acute treatment is indicated.

Background/Purpose: Perampanel (PER) is a selective noncompetitive antagonist of AMPA-glutamate receptors on postsynaptic neurons. PER is used as add-on therapy for the treatment of partial and generalized tonic-clonic seizures in patients older than 12 years of age.

Introduction: Headache in children is one of major causes for consultation in neuropediatric outpatient clinic. The estimated mean prevalence of headache in children and adolescents is ~54%. This study aims to evaluate the outpatient workup in the neuropediatric clinic of Innsbruck in a 2-year period.

Background: Landau-Kleffner syndrome is an epileptic encephalopathy which occurs predominantly in normally developed children aged between 3 and 8 years old. The main symptoms are auditory agnosia, followed by regression of active speech (paraphasia, clipping), at least a complete aphasia, an EEG with epileptic characteristics /− clinical seizures. The EEG patterns show frequent temporoparietal activity, activated in slow-wave sleep. Before or during puberty seizures and EEG-abnormalities disappear. Language impairment may persist although the EEG-abnormalities disappear. Poor prognosis includes aphasia occurring in infancy and continuation of language impairment for longer than 3 years.

Background: Neuromyelitis optica (NMO) is a rare degenerative disorder of the central nervous system affecting mainly optic nerves and spinal cord. It often leads to devastating visual and motor disabilities with a 5-year mortality of 30%; so early recognition and treatment are important. Obligatory criteria for diagnosis are transverse myelitis and optic neuritis in the absence of defining criteria for multiple sclerosis and ADEM. Aquaporin-4-receptor antibody seropositivity is diagnostic as it has been detected exclusively in sera of patients with NMO and neuromyelitis spectrum disorders (NMOSD). In children, presentation may differ from adult onset NMO and is characterized by frequent brain involvement.