Add comprehensive articles on Vascular Dementia and Wallerian Degeneration
- Created a detailed article for Vascular Dementia covering key facts, terminology, imaging findings, differential diagnoses, pathology, clinical issues, and diagnostic checklist. - Developed an extensive article on Wallerian Degeneration including key facts, terminology, imaging features, differential diagnoses, pathology, clinical issues, and diagnostic checklist.
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title: "Epilepsy, Child"
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docid: "a342e5b5-5b98-4003-a437-6d42a483b40e"
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breadcrumbs:
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- "Brain"
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- "Differential Diagnosis"
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- "Brain Parenchyma, General"
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- "Clinically Based Differentials"
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- "Epilepsy, Child"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- Generalized seizure disorders usually nonlocalizing
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- Partial complex (focal) epilepsy usually due to focal structural abnormality [i.e., focal cortical dysplasia (FCD)]
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- Correlate with EEG results
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- High-resolution & 3T MR helpful for subtle lesions
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- 1-mm isotropic T1 for gray matter evaluation
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- 3D FLAIR imaging helpful for identifying FCD
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- PET & SPECT are often complimentary to MR in identifying seizure focus prior to surgical intervention
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- PET: Decreased interictal metabolism in seizure focus
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- SPECT: Increased ictal blood flow in seizure focus
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- ## Helpful Clues for Common Diagnoses
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- **Idiopathic Epilepsy**
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- No structural cause found on MR
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- Generalized: May be inherited
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- Partial: Partial motor seizures, may resolve by puberty
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- **Acquired Causes**
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- Febrile seizure: Most common cause of seizure in children < 5 years
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- Simple febrile seizure < 15 minutes without recurrence does not require imaging
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- Trauma, remote stroke, or infection results in encephalomalacia &/or gliosis, which may cause epilepsy
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- Benign & malignant tumors
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- Toxic, metabolic, & drug abuse
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- **Vascular Malformation**: Arteriovenous & cavernous malformations with hemorrhage
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- **Mesial Temporal Sclerosis**
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- Most common cause of intractable temporal lobe epilepsy in adults
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- 2-hit hypothesis suggests initial injury with inherent vulnerability to neuronal injury
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- Hippocampal atrophy & gliosis
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- May see ipsilateral mammillary body & forniceal atrophy
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- Look for associated FCD, especially in the ipsilateral temporal lobe (FCD type IIIa)
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- **Migrational Anomalies**
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- **Focal cortical dysplasia**
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- Newest classification Blumcke et al 2011
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- Type I: Mild blurring of gray-white junction with mild increased T2 signal of subcortical white matter
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- More common in temporal lobes, difficult to detect
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- Type II: Moderate blurring of gray-white junction & increased T2 signal of subcortical white matter
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- Typically frontal lobes
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- Type IIb includes more dysmorphia & balloon cells: Highly associated with transmantle sign & easier to detect on MR
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- Transmantle sign: T2-hyperintense comet tail from ventricle to cortex; best seen on FLAIR
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- Type III: Associated with another lesion: Mesial temporal sclerosis, tumor, vascular malformation, acquired injury
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- **Polymicrogyria**
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- Small, pebbly, cobblestone, or micronodular-appearing gyri
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- Common migrational malformation with heterogeneous causes
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- TORCH infection (particularly CMV) is common cause of polymicrogyria & seizures
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- Diffuse or bilateral polymicrogyria more likely genetic/syndromic
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- **Heterotopic gray matter**
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- Gray matter nodules within deep white matter follow gray matter signal on all MR sequences
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- Subependymal most common location
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- Can be found incidentally in patients without seizures
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- Diffuse subependymal heterotopia is X-linked
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- **Schizencephaly**
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- Cleft extending from cortical surface to ventricular ependyma, gray matter lined
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- Outpouching or "dimpling" of lateral ventricular contour "points" to cleft
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- May be unilateral or bilateral
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- Open lipped: CSF in cleft; commonly bilateral
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- Closed lipped: No CSF with apposed walls, usually unilateral
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- **Septo-Optic Dysplasia Plus Syndrome**
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- Septum pellucidum absence + optic nerve hypoplasia ± pituitary dysfunction
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- When SOD is associated with schizencephaly &/or polymicrogyria, it is referred to as SOD Plus
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- **Tuberous Sclerosis Complex**
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- Burden of cortical dysplasias (i.e., tubers) correlates with seizure burden
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- T2-hyperintense cortical/subcortical tubers: Similar imaging to type IIb FCD
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- Cortical tubers also similar in histology to FCD type IIb with balloon cells
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- Subependymal nodules can enhance & calcify
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- 10-15% develop subependymal giant cell astrocytoma at foramen of Monro
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- ## Helpful Clues for Less Common Diagnoses
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- **Cortically Based Glioneuronal Tumors**
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- Associated cortical dysplasia with tumor common (type IIIb)
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- **Ganglioglioma**
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- Most common cause of tumor-associated temporal lobe epilepsy
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- Cystic/solid cortically based mass
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- Ca⁺⁺ (~ 50%); enhancement (~ 50%)
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- Temporal lobe most common site
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- **Dysembryoplastic neuroepithelial tumor**
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- Discrete T2-hyperintense "bubbly" cortical mass
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- Medial temporal lobe most common
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- Enhancement may occur (~ 10%) but is less common than ganglioglioma
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- **Holoprosencephaly**
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- Spectrum of failure of cleavage of midline cerebral hemispheres & telencephalon from diencephalon
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- Monoventricle due to absence of septum pellucidum
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- Complete to partial absence of other midline structures: Falx & corpus callosum
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- Fusion of fornices, thalami, & basal ganglia
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- Incomplete separation of frontal lobes
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- More severe cases may include large dorsal cyst
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- **Hemimegalencephaly**
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- Unilateral hemispheric overgrowth
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- Dysplastic enlarged ipsilateral ventricle
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- Associated with genetic/syndromic diseases
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- **Sturge****-****Weber Syndrome**
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- Unilateral trigeminal (V1 & V2) distribution facial port-wine stain
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- Ipsilateral malformation of cortical & pial veins = leptomeningeal enhancement
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- Ipsilateral enlarged choroid plexus, hemiatrophy late finding
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- Gyriform Ca⁺⁺ increases over time
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- **Status Epilepticus**
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- Seizure > 5 minutes or > 1 seizure within 5-minute period
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- Higher likelihood for irreversible brain damage
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- 1/2 are associated with known history of epilepsy
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- Increased T2 signal of predominantly cortex with swelling & possible decreased diffusion
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- ## Helpful Clues for Rare Diagnoses
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- **Lissencephaly Type 1: Subcortical Band Heterotopia**
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- "Smooth" brain lacking normal gyri; thick cortex
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- Can see subcortical smooth gray matter band in many cases
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- LIS1: Posterior predilection of lissencephaly
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- DCX (double cortex): X-linked
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- Females: Primarily diffuse band heterotopia
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- Males: Diffuse lissencephaly, more severe phenotype
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- **Lissencephaly Type 2**
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- Congenital muscular dystrophy: Walker-Warburg, Fukuyama, & muscle-eye-brain
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- Diffuse polymicrogyria (cobblestone lissencephaly) particularly frontal lobes & sylvian fissures
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- Cerebellar polymicrogyria, cysts, vermian hypoplasia, hypomyelination, & eye abnormalities can be seen
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- **Rasmussen Encephalitis**
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- Likely autoimmune inflammation of unilateral cerebral hemisphere
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- Typically at least mesial temporal lobe & insula affected
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- Hemiatrophy late
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