KKI Neuropsych board prep and fun facts

What predicts reading fluency?

Rapid automatic naming

What is a laughing seizure called?

gelastic seizure— involves sudden bursts of laughter, often inappropriate and uncontrollable.

Gelastic seizures are often associated with….

temporal lobe epilepsy or Hypothalamic hamartomas.

Auras are typically associated with

temporal lobe epilepsy

What % do you want to be below to diagnose SLD

the 9th percentile (below an SS of 80)

VCI on the WISC and WAIS are correlated with PPVT-5 at ________

0.84-0.86 correlation

Children with dyslexia most often have ____ type of spelling errors.

Dysphonetic spelling errors. They will have above a 30-40% dysphonetic spelling error rate. Dysphonetic errors with consonants are worse than errors with vowels.

What vitamin helps with irritability on Keppra?

Vitamin B6 helps reduce irritability associated with Keppra.

What is the IQ cutoff for being able to learn to read?

SS of 50-55 is the IQ cutoff for learning to read.

Formerly known as benign childhood epilepsy with centrotemporal spikes (BECTS or BCECTS)

SeLECTS (Self Limited Epilepsy with Centro Temporal Spikes)

Formerly known as benign rolandic epilepsy

SeLECTS (Self Limited Epilepsy with Centro Temporal Spikes)

Formerly known as early onset benign occipital epilepsy

Self LImited Epilepsy with Autonomic Seizures (SeLEAS)

formly known as Panayiotopoulos syndrome

Self LImited Epilepsy with Autonomic Seizures (SeLEAS)

formerly known as Late onset (benign) occipital epilepsy or idiopathic childhood occipital epilepsy–Gastaut type

Childhood Occipital Visual Epilepsy (COVE)

formerly known as Idiopathic photosensitive occipital lobe epilepsy

Photosensitive Occipital Lobe Epilepsy (POLE)

What are the four types of childhood self limited epilepsy syndromes

Selects, Seleas, COVE, POLE

Childhood self-limited epilepsy syndromes normally share these features

No significant structural lesion of brain

birth, neonatal, and antecedent history is usually normal

remission by puberty

normally responsive to anti-seizure meds

EEG abnormalities often activivated in sleep

EEG has normal background

Most frequently childhood self limited epilepsy

SELECTS

Age of onset is between 4-10 years of age, with peak at 7 years old. What epilepsy syndrome is this?

Selects

This epilepsy syndrome is typically associated with focal language and/or reading impairment. OTherwise, cognition is normal.

SELECTS

The characteristic ictal symptoms correspond to the origin of seizures in the rolandic or perisylvian sensorimotor cortex, which represents the face and oropharynx, and include facial numbness or twitching, guttural vocalizations, hypersalivation, drooling, dysphasia, and speech arrest

SeLECTS

self limited epilepsy syndrome presents at a mean age of younger than 5 years (range 1 to 14 years)

SeLEAS

Most common unique seizure type with SeLEAS

1. Vomiting

2. Pallor/flushing/cyanosis

3. Syncope-like seizures

4. Mydriasis (dilation of pupils)

5. Miosis (constricted pupils)

6. Incontinence

7. Coughing

8. Hypersalivation

This epilepsy syndrome has a mean age of presentation of 8 to 9 years (range 3 to 16 years).

COVE

Seizures often include visual symptoms (blindness or hallucinations)

COVE

Seizures for ______ are usually nocturnal and last more than 5 minutes.

SeLEAS

_______seizures are shorter and more frequent compared to SeLEAS seizures

COVE

Most seizures occur during the daytime for this childhood epilepsy syndrome

COVE

Formerly known as fifth day fits (because of their peak day of onset)

Self-limited (familial) neonatal epilepsy (SeLNE)

Formerly known as Benign familial neonatal epilepsy, Benign neonatal convulsions, Benign idiopathic neonatal seizures

Self-limited (familial) neonatal epilepsy (SeLNE)

This epilepsy syndrome usually occurs within the first 7 days of life, with 90% occurring between days 4 and 6

Self-limited (familial) neonatal epilepsy (SeLNE)

Infants with this seizure syndrome have seizures that typically resolve within 6 weeks to 6 months. 30% will develop epilepsy later in life

Self-limited (familial) neonatal epilepsy (SeLNE)

MRIs for Self-limited (familial) neonatal epilepsy (SeLNE) are typically _______. Back EEG is typically ______.

nonlesional; normal; normal MRI is required to confirm the diagnosis

What is development like for infants with Self-limited (familial) neonatal epilepsy (SeLNE)?

Development is typically normal

formerly called benign familial (and nonfamilial) infantile seizures.

Self-limited (familial) infantile epilepsy (SeLIE)

Seizures for this syndrome typically begin between ages 3 and 20 months, with a peak incidence at approximately 6 months of age

Self-limited (familial) infantile epilepsy (SeLIE)

What does the MRI show for Self-limited (familial) infantile epilepsy (SeLIE) ?

Nonlesional; normal

In this epilepsy syndrome, seizures typically abate by the age of one year, and psychomotor development is normal.

Self-limited (familial) infantile epilepsy (SeLIE)

Now the combined name for Ohtahara syndrome and early myoclonic encephalopathy (EME)

Early infantile developmental and epileptic encephalopathy (EIDEE)

onset for this seizure syndrome is within the first 3 months of life. at onset, neurologic exam is abnormal. developmental delays are apparent.

Early infantile developmental and epileptic encephalopathy (EIDEE)

Patients with this epilepsy syndrome early in infancy often have seizures that respond what way to medications

intractable

This infant epilepsy syndrome is associated with severe intellectual disability, reduced life expectancy, treatment-resistant seizures, and potential to evolve to another seizure

Early infantile developmental and epileptic encephalopathy (EIDEE)

previously known as malignant migrating partial seizures of infancy.

Epilepsy of infancy with migrating focal seizures

characterized by drug-resistant migrating focal seizures and developmental delay

Epilepsy of infancy with migrating focal seizures

The hallmark is focal seizures that migrate from one hemisphere or cortical region to another within a single seizure [6]. Onset is within the first year of life, usually within the first six months (mean three months) [58-60]. Seizures are often prolonged, resistant to therapy, and commonly increase in frequency over weeks to months after epilepsy onset. Episodes of status epilepticus are frequent

Epilepsy of infancy with migrating focal seizures (EIMFS)

Drug-resistant epilepsy, severe developmental delay and regression are characteristic of _____________, along with a reduced life expectancy. Some children will also have an associated gut dysmotility and a movement disorder

Epilepsy of infancy with migrating focal seizures (EIMFS)

combined West Syndrome and infants with infatile spasms

Infantile epileptic spasms syndrome — IESS

the triad of epileptic spasms, arrest of psychomotor development, and hypsarhythmia

Infantile epileptic spasms syndrome — IESS

previously known as severe myoclonic epilepsy of infancy

Dravet Syndrome

previously known as benign myoclonic epilepsy in infancy.

Myoclonic epilepsy in infancy (MEI)

myoclonic seizures begin between the ages of four months and three years. The peak onset is from 6 to 18 months [6]. There is a 2:1 male-to-female predominance. Development is usually normal prior to seizure onset, although mild developmental abnormalities may be present

Myoclonic epilepsy in infancy (MEI)

Describe the neurologic exam and MRI for Myoclonic epilepsy in infancy (MEI). Describe development after resolution too

The neurologic examination is normal. MRI typically normally; Most patients have normal neurodevelopmental outcomes

Myoclonic seizures usually resolve spontaneously within six months to five years from the onset of _______________, and most children are able to stop antiseizure medication

Myoclonic epilepsy in infancy (MEI)

also known as infantile spasms; associated with the electroencephalographic (EEG) pattern of hypsarhythmia, and also developmental regression

Infantile epileptic spasms syndrome (IESS)

This antiseizure medication is NOT SAFE for pregnancy

Vimpat

This antiseizure medication is the gold standard for pregnant women

lamictal

What is prognosis for infantile epileptic spasms syndrome (IESS)

guarded; the mortality rate varies from 3 to 33%, with the most important risk factor being significant respiratory system comorbidity. Other adverse outcomes include epilepsy, which may be intractable, and moderate to severe neurodevelopmental disability. Approximately 25 percent of children with infantile spasms have a favorable long-term outcome with seizure freedom and good cognitive outcomes; favorable prognostic factors included early spasm recognition and treatment, short duration of hypsarhythmia, early treatment of spasm relapses or multifocal epileptic discharges on EEG, and prompt management of adverse effects.

Epilepsy syndrome associated with SCN1A pathogenic variant. It is a rare genetic epilepsy and a developmental and epileptic encephalopathy

Dravet Syndrome

Core features include refractory epilepsy characterized by multiple different seizure types, neurodevelopmental delay and neurologic disability that begin after seizure onset, and cognitive and motor system dysfunction persisting into adulthood

Dravet Syndrome

classified as a combined generalized and focal epilepsy, with both generalized and focal seizures

Dravet Syndrome

Seizure onset most often occurs within the first year of life, usually between five and eight months (range 1 to 21 months), in a previously healthy infant . As a rule, infants with _______ have had normal physical and psychomotor development at the time of their first seizure. First seizure is typically tonic clonic in context of fever.

Dravet Syndrome

Neurodevelopmental impairment typically begins shortly after seizure onset within the first year of life and becomes progressively evident from the second year onward. In most children with _______, developmental impairment is due to stagnation (ie, lack of or slower progression) rather than regression. In some cases, status epilepticus can lead to loss of previously acquired skills. Language tends to emerge at an appropriate age but then progresses slowly, to the extent that many patients fail to reach the stage of constructing elementary sentences.

Dravet Syndrome

Motor system dysfunction is present in most patients with ______ . Characterized by a “crouched gait”, ataxia, tremor, dysarthria, pyramidal signs, parkinsonian signs.

Dravet Syndrome

What is cognitive outcome like in Dravet Syndrome?

Cognitive impairment in the moderate to severe range. Attention, visual motor integration, visual perception, and executive functions tend to be more impaired than language

severe developmental epileptic encephalopathy, in which seizure activity and epileptic discharges cause or contribute to severe cognitive impairment and behavioral disorders beyond what would be expected from the underlying etiology; onset is in childhood and seizures are treatment resistant.

Lennox-Gastaut syndrome

associated with multiple seizure types; severe cognitive impairment; seizures most prominant in sleep

Lennox-Gastaut syndrome

This language subtest is a strong predictor of specific language impairment in children under 6 years

sentence repetition

The _______ fissure separates the lingual gyrus and the cuneus.

calcarine

Upper halves of visual fields are processed by the gyri below the ______ fissure

calcarine

Lower halves of the visual fields are processed by the gyri above the _____________fissure

calcarine

The calcarine fissure separates these two areas of the brain

lingual gryus and cuneous

Part of thalamas that plays a role in visual processing

LGN; lateral geniculate nucleus

this brain area is responsible for recognizing and processing faces

fusiform face area

“what” visual pathway is the ______ stream and functions in ______

ventral stream; recognizing objects

“where” visual pathway is the ______ stream and functions in _____________

dorsal stream; processing where things are in space

the “specialized movement” stream functions in

visual analysis of movement of body parts and biological objects

This syndrome has these three symptoms: optic ataxia, ocular apraxia, simultanagnosia

Balint’s syndrome

inability to distinguish colors (e.g., hue)

achromatopsia

inability of an individual to visually perceive more than a single object at a time

simultanagnosia

Classically, the inability to identify familiar faces. However, deficit is the inability to identify specific members of a class

prosopagnosia

congenital hydrocephalus is typically caused by _____

aqueductal stenosis or neural tube defect (spina bifida, dandy walker syndrome)

narrowing of the cavity that connects the 3rd and 4th ventricles

aqueductal stenosis

classic landau kleffner symptom

auditory agnosia

neuroanatomical correlated of alexia without agraphia

dominant (left) occipital lobe lesions that involves white matter of the posterior corpus callosum

neuroanatomical correlated of alexia with agraphia

dominant (left) angular gyrus in the inferior parietal lobe

in order for a stroke to be called “perinatal” it has to occur during pregnancy, labor, delivery, or up until the ____ day of life

28th

perinatal strokes typically occur in ______ hemisphere

left

perinatal strokes typically occur in the _______ artery for term infants

MCA

perinatal strokes for term infants results in what kind of damage?

cortical and subcortical

perinatal stroke in preterm infants usually resuults in what kind of damage?

periventricular / white matter damage

newborns whose stroke occurs during labor/delivery typically present with ____

seizures

newborns whose stroke occurred before birth (by a few days or more) may be ______

asymptomatic

degree of _______ has been found to correlated significantly with IQ in children with perinatal strok

hemiplegia

in hemiplegic CP, for patients with large lesions, you see ______ reorganization of the primary motor cortex. The corticospinal tract connects are _____.

ipsilateral; uncrossed

kids with hemiplegic CP, you tend to see _______ movements, specifically in kids with larger lesions and ipsilateral uncrossed corticospinal tract fibers

mirror

the most common type of gait described in CP

circumducting gait

recognition of objects by touch

stereognosis

this cortex has little plasticity

sensory cortex

kids with perinatal stroke show partial thinning of __________, suggesting that the stroke could have secondary effects on the uninjured hemisphere

corpus callosum