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