Secondary Epilepsies Flashcards
VGKC Complex
Symptoms = personality or behavioral changes, myoclonus, neuropathy, and hyponatremia.
Associated: SCLC, thymoma
NMDA Receptor
Psychosis, extrapyramidal d/o, choreoathetosis, dysautonomia
Associated: Ovarian teratoma
GAD
Stiff person syndrome, ataxia, brainstem encephalitis, parkinsonism, and diabetes (T1DM)
Asosciated: Thymoma, Breast adenocarcinoma
Ma1, Ma2
Brainstem encephalitis
Associated: testicular
ANNA-1 (Hu)
Brainstem encephalitis, autonomic or sensory neuropathy
Associated: SCLC
CRMP-5
Dementia, personality change, chorea, ataxia, and neuropathy
SCLC Thymoma
Amphiphysin
Dementia myelopathy, and neuropathy
Associated: SCLC breast adenocarcinoma
If Antibody target is GABAreceptor, symptoms would be:
Encephalopathy
Associated with Cancer
SCLC, thymoma
If antibody target ANNA -2 (Ri), symptoms would be
Brainstem encephalitis, cerebellar ataxia,
Associated cancers SCLC, breast, gynecological
If antibody target AMPA, symptoms would be
Psychiatric
Associated cancer
Multiple solid cancers
Epileptogenic tumors:
DNETs > gangliogliomas > low grade astrocytomas
Cortical Malformations (MCD)
-Hemimeg
-Lissencephaly
-Double cortex syndrome/subcortical band heterotopia
-Polymicrogyria
Perisylvian polymicrogyria sydnrome
Schizencephaly
Porencephalic cyst
-Periventricular nodular heterotopia
Periventricular Lesions between TSC vs PVNH
Subependymal Nodules (TSC) Smaller Less in number Heterogeneous Calcified WM hyperintensity on MRI
PVNH Larger More in number, often bilateral Homgenous Not calcified Gray matter intensity on MRI
Focal Cortical Dysplasia
Neuroimaging findings
Typical MRI findings: None or blurred gray-white junction, thickened cortex, transmantle sign (T2 hyperintensity extending radially from ventricle to cortex)
May found on functional imaging (PET, interictal SPECT)
Focal Cortical Dysplasia (FCDs)
Severity classficiation
Classified pathological severity
- Mild - microdysgenesis
- Type I - intermediate, may not be seen on MRI
- Type II - most severe type, balloon cells IIb on pathology
- Type III - dual pathology (FCD + other lesions)
Post traumatic seizure
Single late unprovoked post-traumatic seizures is nearly synonymous with epilepsy
Seizure recurrence after single late was 86% within two years.
Therefore: one late seizure is necessary to diagnose epilepsy after trauma and should consider AED
PNES
Diagnosis tool
video EEG gold standard for post-traumatic seizure-like events
Posttraumatic Seizure prophylaxis
Strongest evidence for prevention of early seizures in adults, data is insufficient
Tx phenytoin after severe head injury x 1 week, treatment does not reduce mortality, disability or late seizures.
Head trauma classification
Mild: <30 min LOC, no structural brain injury
Moderate: 30min-24hr LOC, skull fx w/ contusion, ICH
Severe: >24hr, brain contusion, ICH, dural penetration
Seizure presenting as stroke:
Percentages
80% neonates
30% children
rare adults
Epilepsy-risk after pediatric stroke
Up to 40% in pediatric stroke
<5% in adults
Post stroke seizures
Early = within first week Late = after 1st week
Risk of further seizures after single late seizure >50% –> AED strongly considered
Predictors of post stroke epilepsy
Cortical location
Presence of hemorrage
Stroke severity (exam and NIHSS)
EEG findings in stroke
Focal slowing or generalized slowing
LPDs- rare, may predict early seizures, but not necessarily later epilepsy
Mesial temporal sclerosis
One of the most common causes of adult onset epilepsy
Also found ~14% without epilepsy
Semiology: abdominal auras (nausea, pressure, butterflies, epigastric rising), fear, unpleasant taste or smell, oroalimentary or ipsilateral automatisms, and autonomic phenomena
MTS Findings:
EEG
MRI
Ictal EEG: anterior temporal rhythmic theta or alpha actvitiy >5Hz within 30 seconds of seizure onset
MRI - hippocampal atrophy*** on T1, hippocampal hyperintesnsity on T2,
PET: temporal hypometabolism
Histopathology of MTS
Neuronal loss and gliosis in CA1, CA3, CA4 hippocampal regions with relative sparing of CA2
Predictors of good surgical outcomes in MTS
Later age at onset Shorter duration of epilepsy Presence of febrile seizures Positive MRI (or PET w/ neg MRI) Unilateral findings on PET Concordant data - matching localization of semiology, EEG, functional imaging, anatomical imaging) Lack of need for intracranial monitoring
Chance of seizure freedom after resection in MTS
60-70%
Surgical options with MTS
selective amygdalohippocampectomy
Tailored temporal lobectomy (sparing eloquet function)
Hippocampal laser ablation
Standard anterior temporal lobectomy
Tumors are more epileptogenic
General features
Adult onset
Lower grade `
Cortical tumors
Tumors closer to sensitive networks - hippocampus, motor cortex (*parietal tumors)
Less likely to cause tumors
High grade, fast growing tumors (ex GBM, primary CNS lymphoma)
Risk of having recurrent/refractory seizures from tumor
If seizures is the initall presenting symptoms of tumor increases risk of refractory seizures and recurrent seizures
AAN recommendations for AED prophylaxis
Strongly AGAINST AED ppx in tumors without hx of seizures
-Can by used peri- or post-operatively x 1 week
Vascular malformations associated with epilepsy
AVMs
Cavernous malformations
(Developmental venous anomalies usually incidental findings are not epileptogenic)
Mechanism of epilepsy in vascular malformations
Surrounding hemorrhage, gliosis, encephalomalacia are the epileptogenic tissues
Surgical management goals for vascular malformations
- Seizure freedom
- Hemorrage prevention
ECOG better outcomes especially in temporal cavernous malformation.
AVM
Direct connection between arteries and veins without capillaries in between
MRI: = small collection of signal void
Cavernous Malformations
aka cavernous angiomas or cavernomas
small bundles of brittle vascular endothelium (not true blood vessels) that lead to recurrent bleeding
MRI = heterogeneous, with core mixed signal intensity surrounded by T2 or GRE hypointense rim
