Secondary Epilepsies Flashcards by Jyes Querubin

VGKC Complex

Symptoms = personality or behavioral changes, myoclonus, neuropathy, and hyponatremia.

Associated: SCLC, thymoma

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NMDA Receptor

Psychosis, extrapyramidal d/o, choreoathetosis, dysautonomia

Associated: Ovarian teratoma

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GAD

Stiff person syndrome, ataxia, brainstem encephalitis, parkinsonism, and diabetes (T1DM)

Asosciated: Thymoma, Breast adenocarcinoma

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Ma1, Ma2

Brainstem encephalitis

Associated: testicular

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ANNA-1 (Hu)

Brainstem encephalitis, autonomic or sensory neuropathy

Associated: SCLC

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CRMP-5

Dementia, personality change, chorea, ataxia, and neuropathy

SCLC Thymoma

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Amphiphysin

Dementia myelopathy, and neuropathy

Associated: SCLC breast adenocarcinoma

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If Antibody target is GABAreceptor, symptoms would be:

Encephalopathy
Associated with Cancer
SCLC, thymoma

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If antibody target ANNA -2 (Ri), symptoms would be

Brainstem encephalitis, cerebellar ataxia,

Associated cancers SCLC, breast, gynecological

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If antibody target AMPA, symptoms would be

Psychiatric
Associated cancer
Multiple solid cancers

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Epileptogenic tumors:

DNETs > gangliogliomas > low grade astrocytomas

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Cortical Malformations (MCD)

-Hemimeg
-Lissencephaly
-Double cortex syndrome/subcortical band heterotopia
-Polymicrogyria
Perisylvian polymicrogyria sydnrome
Schizencephaly
Porencephalic cyst
-Periventricular nodular heterotopia

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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

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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)

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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)

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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

1. Seizure freedom 2. 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