CNS Conditions with EEG Findings

This comprehensive slideshow on Pediatric CNS Conditions with EEG Findings is an invaluable resource for medical students and healthcare professionals specializing in neurology, pediatrics, or related fields. It provides a detailed overview of various pediatric neurological conditions, categorized into distinct groups such as epileptic encephalopathies, neonatal seizure disorders, genetic/metabolic disorders, and more. Each condition is accompanied by specific EEG findings and clinical notes, offering a clear understanding of the diagnostic patterns and key features. The interactive format allows users to navigate through the content seamlessly, making it an excellent tool for both learning and quick reference in clinical settings.

For medical students, this resource serves as a structured guide to understanding the correlation between clinical presentations and EEG patterns, which is crucial for diagnosing and managing pediatric neurological disorders. For professionals, it acts as a quick refresher or a reference during patient evaluations, especially when dealing with rare or complex cases. The inclusion of rare conditions and ultra-rare genetic epilepsies ensures that even experienced practitioners can benefit from the depth of information provided. By combining theoretical knowledge with practical insights, this slideshow bridges the gap between academic learning and real-world clinical practice, making it an essential tool for anyone involved in pediatric neurology.

Epileptic Encephalopathies

West Syndrome (Infantile Spasms)

Hypsarrhythmia pattern: High-amplitude, irregular, chaotic slow waves with multifocal spikes
Modified hypsarrhythmia variants may occur

Typically presents between 3-12 months; urgent treatment required

Lennox-Gastaut Syndrome

Slow spike-and-wave complexes (< 2.5 Hz)
Paroxysmal fast rhythms during sleep
Background slowing

Usually develops between 3-5 years of age

Dravet Syndrome

Initially normal background
Generalized spike-waves and polyspike-waves
Photosensitivity often present

Associated with SCN1A mutations; onset in first year of life

Neonatal Seizure Disorders

Benign Familial Neonatal Epilepsy

Discontinuous background pattern
Focal sharp waves
Rhythmic alpha/theta bursts

KCNQ2/KCNQ3 mutations; generally good prognosis

Ohtahara Syndrome

Suppression-burst pattern: High-voltage bursts alternating with nearly flat suppression periods
Pattern present in both sleep and waking states

Early infantile epileptic encephalopathy; poor prognosis

Genetic/Metabolic Disorders

GLUT1 Deficiency Syndrome

2.5-4 Hz spike-wave discharges
Focal and multifocal epileptiform discharges
Background slowing post-prandially

Responds to ketogenic diet; SLC2A1 gene mutations

Nonketotic Hyperglycinemia

Burst-suppression pattern
Multifocal spikes
Periodic patterns

Often presents in neonatal period; poor prognosis

Pyridoxine-Dependent Epilepsy

High-amplitude rhythmic delta activity
Burst-suppression pattern may occur
Continuous spike-wave activity

Diagnostic trial of pyridoxine may be therapeutic

Autoimmune/Inflammatory Conditions

Anti-NMDA Receptor Encephalitis

Extreme delta brush pattern
Diffuse slowing
Disorganized background

More common in females; may have psychiatric symptoms

Rasmussen Encephalitis

Unilateral slowing and epileptiform discharges
Progressive deterioration of background
Periodic lateralized epileptiform discharges (PLEDs)

Progressive unilateral hemisphere dysfunction

Childhood Epilepsy Syndromes

Childhood Absence Epilepsy

3 Hz spike-and-wave discharges
Normal background activity
Hyperventilation typically triggers absences

Peak onset 4-8 years; generally good prognosis

Benign Epilepsy with Centrotemporal Spikes (BECTS)

High-voltage centrotemporal spikes
Normal background activity
Activation during drowsiness and sleep

Self-limited; resolves by adolescence

Structural Brain Disorders

Sturge-Weber Syndrome

Asymmetric background activity
Focal slowing over affected hemisphere
Focal epileptiform discharges

Associated with leptomeningeal angiomas

Tuberous Sclerosis Complex

Multifocal epileptiform discharges
Hypsarrhythmia may occur in infancy
Background may be normal or show focal slowing

Multiple seizure types may occur

Infectious/Post-infectious Conditions

Herpes Simplex Encephalitis

Periodic lateralized epileptiform discharges (PLEDs)
Focal temporal slowing
Background disorganization

Requires immediate antiviral treatment

Subacute Sclerosing Panencephalitis

Periodic high-amplitude complexes (Radermecker complexes)
Progressive background deterioration

Late complication of measles infection

Status Epilepticus Variants

Electrical Status Epilepticus during Sleep (ESES)

Continuous spike-waves during slow-wave sleep
Spike-wave index >85% during NREM sleep

Associated with cognitive regression

Febrile Infection-Related Epilepsy Syndrome (FIRES)

Evolution from focal to diffuse slowing
Multifocal epileptiform discharges
Status epilepticus patterns

Devastating condition with poor prognosis

Miscellaneous Disorders

Angelman Syndrome

High-amplitude 2-3 Hz rhythmic delta
Spikes/sharp waves
Characteristic pattern of notched delta waves

UBE3A gene mutations/deletions

Rett Syndrome

Loss of normal sleep characteristics
Multifocal spike discharges
Generalized slowing with age

MECP2 mutations; predominantly affects females

Mitochondrial Disorders

MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes)

Focal slowing in stroke-like lesions
Periodic lateralized epileptiform discharges
Multifocal spike-wave complexes

MT-TL1 gene mutations; progressive course

MERRF (Myoclonic Epilepsy with Ragged Red Fibers)

Generalized spike-wave and polyspike-wave discharges
Photoparoxysmal response
Progressive background slowing

MT-TK gene mutations; progressive myoclonus

Alpers-Huttenlocher Syndrome

High-amplitude delta with polyspikes
Multifocal spike-wave discharges
Progressive background deterioration

POLG mutations; avoid valproate

Channelopathies

SCN2A-Related Disorders

Multiple seizure patterns
Focal and multifocal epileptiform discharges
Background may be normal or show slowing

Age-dependent phenotypes; sodium channel mutation

KCNQ2 Encephalopathy

Burst-suppression pattern
Multifocal epileptiform discharges
Background slowing

More severe than benign familial neonatal seizures

Progressive Myoclonic Epilepsies

Lafora Disease

Generalized polyspikes and waves
Photosensitivity
Progressive background slowing
Occipital seizures with visual symptoms

EPM2A/EPM2B mutations; adolescent onset

Unverricht-Lundborg Disease

Generalized spike-wave and polyspike discharges
Photosensitivity
Background may show mild slowing

CSTB gene mutations; better prognosis than other PMEs

Neurocutaneous Syndromes

Neurofibromatosis Type 1

Usually normal background
Focal epileptiform discharges if tumors present
Sleep architecture may be disturbed

NF1 gene mutations; variable epilepsy risk

Incontinentia Pigmenti

Multifocal spike-wave discharges
Background slowing over affected regions
Possible burst-suppression in severe cases

NEMO gene mutations; predominantly affects females

Chromosomal Disorders

Ring Chromosome 20 Syndrome

Long runs of frontal theta activity
Nocturnal seizure patterns
Prolonged high-amplitude delta waves

Characteristic nocturnal seizures; drug-resistant epilepsy

1p36 Deletion Syndrome

Multifocal spike-wave discharges
Asymmetric background activity
Possible hypsarrhythmia

Most common terminal deletion syndrome

Metabolic Disorders

Gaucher Disease Type 2

Polyspike pattern
Background slowing
Electrodecremental events

GBA gene mutations; severe infantile form

Creatine Deficiency Syndromes

Generalized spike-wave discharges
Multifocal epileptiform abnormalities
Background slowing

Includes GAMT, GATM, and SLC6A8 deficiencies

Malformations of Cortical Development

Lissencephaly

High-amplitude beta activity
Multifocal epileptiform discharges
Background disorganization

Various genetic causes including LIS1, DCX

Polymicrogyria

Focal or multifocal epileptiform discharges
Fast rhythms over affected regions
Variable background changes

Can be focal or diffuse; multiple genetic causes

Ultra-Rare Genetic Epilepsies

CDKL5 Deficiency Disorder Ultra-Rare

Early-onset epileptic encephalopathy pattern
Multifocal epileptiform abnormalities
Pseudo-periodic generalized spike-waves
Distinctive delta-brush pattern in early infancy

X-linked; predominantly affects females; treatment-resistant

PCDH19-Related Epilepsy Ultra-Rare

Focal and multifocal sharp waves
Normal background initially
Seizure clusters with focal onset
Variable interictal patterns

Female-specific epilepsy; clustering seizures characteristic

CHD2 Encephalopathy Ultra-Rare

Generalized spike-wave discharges
Marked photosensitivity
Myoclonic-atonic seizure patterns
Background slowing may be progressive

Prominent photosensitivity; myoclonic seizures

Rare Metabolic Epilepsies

Molybdenum Cofactor Deficiency Ultra-Rare

Burst-suppression pattern
Multifocal epileptiform discharges
Background discontinuity
Status epilepticus patterns

Neonatal onset; MOCS1/MOCS2/GPHN genes

Cerebrotendinous Xanthomatosis Ultra-Rare

Focal temporal sharp waves
Progressive background slowing
Periodic lateralized patterns
Photoparoxysmal responses

CYP27A1 mutations; treatable with chenodeoxycholic acid

Menkes Disease Ultra-Rare

Hypsarrhythmia variants
Multifocal spike discharges
Profound background slowing
Evolution to burst-suppression

ATP7A mutations; copper transport disorder

Rare Developmental Epilepsies

FOXG1 Syndrome Ultra-Rare

High-amplitude fast activity
Multifocal epileptiform abnormalities
Poor organization of sleep patterns
Variable background slowing

Congenital variant of Rett syndrome

DHDDS-Related Epilepsy Ultra-Rare

Generalized spike-wave discharges
Focal epileptiform abnormalities
Progressive background changes
Sleep-activated discharges

Recently described developmental epileptic encephalopathy

Rare Neurodegenerative Epilepsies

Neuronal Ceroid Lipofuscinosis Type 2 Ultra-Rare

Occipital spikes with photic stimulation
Progressive background slowing
Giant visual evoked potentials
Loss of sleep architecture

TPP1 deficiency; enzyme replacement available

Sialidosis Type 1 Ultra-Rare

Polyspike-wave complexes
Progressive background disorganization
Enhanced photoparoxysmal response
Myoclonic seizure patterns

NEU1 mutations; progressive myoclonus

Rare Immune-Mediated Epilepsies

GAD65 Antibody-Associated Epilepsy Ultra-Rare

Temporal lobe epileptiform discharges
Bitemporal independent spikes
Status epilepticus patterns
Variable background changes

Associated with other autoimmune conditions

GABABR Encephalitis Ultra-Rare

Status epilepticus patterns
Diffuse slowing
Multifocal epileptiform discharges
Periodic patterns in severe cases

May be paraneoplastic; status epilepticus common

Complex Structural Epilepsies

Hypothalamic Hamartoma Rare

Normal background activity
Focal epileptiform discharges
Gelastic seizure patterns
Secondary generalization patterns

Characteristic gelastic seizures; surgical options available

Hemihydranencephaly Ultra-Rare

Markedly asymmetric background
Absence of activity over affected hemisphere
Contralateral epileptiform discharges
Reorganized sleep patterns

Extreme variant of hydranencephaly