Rasmussen syndrome

Rasmussen syndrome is a rare inflammatory brain disorder, usually beginning in childhood, that causes drug-resistant seizures and progressive injury to one cerebral hemisphere.

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Focal seizures: Seizures that begin in networks confined to one cerebral hemisphere, with or without impaired awareness. Seizures often arise from the affected hemisphere and may become frequent or continuous.
Electroencephalography: A method for recording electrical activity from the brain through electrodes placed on the scalp or other surfaces. EEG can reveal focal epileptic activity and guide assessment of persistent seizures.
Drug-resistant epilepsy: Epilepsy that persists despite adequate trials of two appropriately chosen and tolerated antiseizure medication regimens. Medication resistance is a defining clinical problem in Rasmussen syndrome.
Focal cortical dysplasia: A developmental malformation of the cerebral cortex that can cause focal, drug-resistant epilepsy. It can cause similar seizures but is a structural developmental abnormality rather than progressive inflammation.
Theodore Rasmussen: A Canadian neurosurgeon and epileptologist known for describing a chronic focal encephalitis associated with intractable seizures. His clinical and pathological work established the syndrome’s defining pattern.
Epilepsia partialis continua: A form of focal motor status epilepticus marked by persistent or recurring jerking in a body part. This distinctive seizure pattern can accompany Rasmussen syndrome and resist medication.
Magnetic resonance imaging: An imaging method that uses magnetic fields and radio waves to create detailed images of the body's internal structures. MRI helps track inflammation-associated signal changes and progressive hemispheric atrophy.
Cerebral hemisphere: One of the two major halves of the cerebrum, connected by neural pathways including the corpus callosum. The syndrome progressively affects one of these two halves.
Sturge-Weber syndrome: A congenital disorder involving a facial capillary malformation and abnormal blood vessels in the brain and eye. It can cause unilateral seizures and brain atrophy, but has a distinct vascular and congenital basis.
Chronic encephalitis and epilepsy: The 1958 report by Theodore Rasmussen and colleagues describing chronic focal encephalitis with intractable seizures. This report introduced the clinical entity later known as Rasmussen syndrome.