Neurology Part 2 Flashcards by Denisse Tiangco

transient occurence of signs or symptoms due to abnormal excessive or synchronous neuronal activity

Seizure

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2 or more unprovoked seizure

Risk of recurrent seizure due to chronic underlying process

Clinical phenomenon not disease

R/o trauma, infection, tumor, stroke, metabolic

Epilepsy

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ILAE commission on classification and terminology

Clinical feature

EEG

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Originate within networks limited to one brain region

Assoc with structural abnormalities of brain

Further described as intact or impaired awareness, motor or nonmotor, focal to bilateral tonic clonic

May evolve into generalized prev called focal with 2 generalization the new rely on type of gen seizure evolving from focal seizure

Focal

Partial no longer used.

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Arise within and rapidly engage networks distributed across both cerebral hemisphere

Result from cellular, biochemical or structural abnormality with widespread distribution

Motor (tonic-clonic, atonic, myoclonic)
Nonmotor (absence)

Generalized seizure

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New classification emphasizes on

Awareness (intact or impaired)

Nature of onset (motor or nonmotor)

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Focal with intact awareness may be

Motor (tonic, clonic, myo)

Nonmotor (sensory, autonomic, emotional) without awareness impairment

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Spread of activity over a progressively larger region of motor cortex

Jacksonian march - focal motor

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Localized paresis for minutes to many hours in involved region following seizure

Todd’s paralysis - focal motor

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Seizure continuing for hours or days

Epilepsia partialis continua - focal motor

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Focal seizure manifestation

Somatic sensation (paresthesia)
Visual (flashing light or formed hallucination)
Equilibrium (sensation of falling or vertigo)
Autonomic dysfunction (flushing, sweating and piloerection)
Temporal or frontal (hearing, olfaction, emotional state)
Unusual intense odor (burning rubber, kerosene), sounds, epigastric from stomach or chest
Odd internal feeling, de ja vu, illusion of object appearing smaller (micropsia) or macropsia

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Focal seizures with impaired awareness or unable to respond begin with

aura

Unable to respond to commands during seizure with impaired recollection of awareness of ictus

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During start of ictal phase, the patient is in

motionless stare marking onset of impaired awareness

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Focal seizures with impaired awareness also may come with

automatism (involuntary automatic behavior)
chewing
lip smacking
picking movement of hand

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Sudden brief lapses of consciousness WITHOUT loss of postural control

Lasts for seconds

Consciousness returns as suddenly as it was lost

No postictal confusion

Main seizure type in 15-20% of children with epilepsy

Typical absence

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Absence symptoms

Rapid blinking of eyelid
Chewing
Small amplitude clonic movement of hands

Childhood 4-10 years or early adolescence

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First clue to absence is unexplained

daydreaming and decline in school performance

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Absence electrophysiological hallmark

Provoked by

generalized, symmetric, 3 Hz spike and slow wave discharge beginning and ending suddenly superimposed on normal EEG background

Hyperventilation

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Main seizure type in 10% of all persons with epilepsy

Most common type from metabolic derangement

Abrupt without warning

Initial tonic contraction

Generalized tonic-clonic seizure

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Tonic contraction of muscle of expiration and larynx at onset will produce a loud moan

ictal cry

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Impaired respiration
Secretions pool in oropharynx
Cyanosis

Contaction of jaw ms cause biting of tongue

Marked enhancement of sympathetic tone leading to inc in heart rate, blood pressure and pupillary size

Generalized tonic-clonic seizure

Tongue dep at bedside

Sidelie while lifting chin

It should stop in 2-3 mins

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After 10-20sec

Superimposition of ms relaxation on tonic ms contraction

Lasts no more than 1 minute

Post ictal phase: unresponsiveness, muscular flaccidity, excessive salivation, bladder and bowel incontinence

Clonic phase

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Sudden and brief ms contraction involving one or entire part of the body

Normal physiologic form of myoclonus is sudden jerking movement observed while falling asleep

Myoclonic seizure

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Pathologic myoclonus seen in

EEG shows

Metabolic d/o
Degenerative CNS
Anoxic brain

Bilaterally synchronous spike and slow wave

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Briefly sustained flexion or extension of proximal or truncal muscle EEG shows

Epileptic spasm Hypsarrhythmia - diffuse giant slow wave with chaotic background of irregular, multifocal spikes and sharp waves

Disorders in which epilepsy is predominant feature with evidence (clinical, EEG, Radio, genetic) Suggesting common underlying mechanism

Epilepsy syndrome Juvenile myoclonic epilepsy Lennox Gastaut syndrome

Generalized seizure of unknown cause Early adolescence Bilateral myoclonic jerk may be single or repetitive Myoclonic seizure most frequent in morning after awakening and provoked by sleep deprivation

Juvenile Myoclonic Epilepsy

Children Triad: Multiple seizures (GTC, atonic and absence) EEG: <3Hz spike and wave discharge Impaired cognitive function

Lennox Gastaut Syndrome

Loss of specific cell population within hippocampus MRI: small hippocampus with inc T2 weighted sequence, small temporal lobe, enlarged temporal horn Aura, behavioral arrest, automatism, unilat posturing, memory loss, dysphasia, post ictal disorientation Hx of febrile seizure, family hx of epilepsy, early onset, rare gen seizure, remit and reappearing seizure, intractable

Mesial Temporal Lobe Epilepsy

Progressive myoclonus epilepsy gene mutation Noncaspase cysteine protease inhibitor Block neuronal apoptosis

Cystatin B

Progressive myoclonus epilepsy Lafora’s disease gene mutation Protein tyrosine phosphatase PTP anti-apoptotic activity

Laforin gene

Classic lissencephaly MR seizure gene mutation

Doublecortin gene

Neonatal period and early infancy cause of seizure

``` Hypoxic ischemic enceph Trauma CNS infection Congenital CNS abnormality Metabolic disorder Cocaine, heroin and ethanol taking mother Hypoglycemia and hypocalcemia Inborn errors of metab Pyridoxine VitB6 deficiency ```

Most common cause of neonatal meningitis

Strep agalactiae E coli Listeria monocytogenes

Fetal alcohol syndrome

thin upper lip | smooth philtrum

Most common seizures arising in late infancy and early childhood occur peaks

febrile seizure between 3 months - 5 years peak at 18 to 24 months

GTC in febrile illness occurs because of

otitis media respiratory infection gastroenteritis

Most common cause of otitis media in children

Strep pneumoniae

Single isolated event, brief and symmetric

Febrile seizure

Repeated seizure activity >15 minutes Focal feature

Complex febrile seizure

Infants >1 mo <12 years

Febrile seizure Genetic disorder CNS infection

Most common cause of seizure in adolescent 12-18

trauma

Most common cause of seizure in adult 18-35

Trauma Alcohol withdrawal Autoantibodies NMDA

Autoantibody directed against CNS antigen such as K ch or glutamate receptor

NMDA enceph

Most common cause of seizure in older adults

``` CVD tumor Alcohol withdrawal Subdural hematoma Autoantibodies ```

Drugs that cause seizure

``` Alkylating (busulfan, chlorambucil) Antimalarial Antimicrobial (b lactam) Quinolone Acyclovir INH Ganciclovir Meperidine Fentanyl Tramadol Ephedra Gingko Cyclosporine Buproprion Clozapine Baclofen withdrawal Amphetamine Flumazenil ```

Busulfan can cause

pulmonary fibrosis also bleomycin

Notorious b lactam for seizure

Imipenem

Tramadol can cause

Seizure and hallucination

Binds to SV2 receptor | Metabolized in kidney

Levetiracetam

Inhibition of sodium dependent action potential

``` Phenytoin Carbamazepine Lamo Topimarate Zonisamide Lacosamide Rufinamide ```

Phenytoin causes is a class I anti arrhythmic

gingival hyperplasia

Topimarate can be used for

migraine

Notorious for causing Steven Johnson Syndrome

``` Phenytoin Carbamazepine Lamotrigine Topimarate Phenobarbital ```

Ataxia | Nystagmus

Phenytoin toxicity

Inhibits voltage gated calcium channels

Phenytoin Gabapentin Pregabalin

Inhibits both Na And voltage gated Ca channels

Phenytoin

Act on calcium channels (gabanoids) by binding to

alpha2 delta subunit

Facilitates opening of K channels

Ezogabine

Attenuates glutamate activity

Lamotrigine also Na inh Topimarate also Na inh Felbamate

Potentiates GABA A receptor

Benzodiazepine | Barbiturates

Increases availability of GABA

Valproic acid Gabapentin Tiagabine

Modulates release of synaptic V2 receptors

Levetiracetam | Brivaracetam

Inhibiting T type Ca channel in thalamic neurons for absence

ethosuxamide | valproic acid

Drop attacks | Loss of postural tone

atonic seizure

Drug of choice for infantile spasm

ACTH

Kepra Levetiracetam is eliminated via

renal

Rhythmic oscillation of body part due to intermittent muscle contraction

Tremor

Involuntary, patterned, sustained, repeated ms contraction | twisting movement and abnormal posture

Dystonia

Slow, distal, writhing involuntary movement with propensity to affect arms and hands dystonia of inc mobility

Athetosis

Rapid semi purposeful graceful dancelike nonpatterned involuntary movement

Chorea

Hypokinetic disorders

Bradykinesia (slowness) Hypokinesia (dec amplitude) Akinesia (absence of movement)

Progressive neurodegenerative disorder | Movement disorder causing significant disability and dec quality of life

Paralysis agitans | PD

Genes in Parkinsons Protein in PD

PINK1 PARKIN gene alpha synuclein

Assoc with juvenile onset PD

pink1

Main neuropathologic feature in PD Eosinophilic round cytoplasmic inclusion

Lewy body

PD features

Hypokinesia Rigidity Resting tremor Postural instability Other motor Craniofacial Hypomimia (masked facie, dec eye blinking) Speech: hypophonia, hypokin dysarthria, dysphagia, sialorrhea Ms: dystonia, stooped posture, camptocormia (severe kyphosis) Gait: shuffling short step gait, freezing and festination

Pull patient back and patient prevents self from falling but fails due to loss of balance

Retropulsion

Inc synthesis of dopamine

L Dopa

Inhibits breakdown of dopamine

Selegiline

Stimulates release of dopa

Amphetamine

Stimulates dopamine receptor sites directly Also prolactinoma

Bromocriptine | Pramipexole

Blocks uptake and enhances release of dopamine Also in influenza

Amantadine

Contraindications for rTPA

Recent surgery BP >180 75-80 y/o Neurologic deficit no more than 5 hrs onset

Antiplatelet of choice for acute ischemic stroke IST antithrombotic drug of study

Aspirin

Cause of delayed neuro deficit in SAH

Vasospasm Hydroceph Rerupture

First and most affected CN in ICP

CN VI CN IV CN III

Abnormal gait Dementia Urinary incontinence

Normal pressure hydrocephalus

Not characteristic of PD

Masked facies with inc blinking

Brainstem, limbic, cortex | Fall with executive and personality changes

Progressive Supranuclear Palsy | Steele-Richardson Olszweki Syndrome

``` Dementia Movement do due to severe atrophy in perirolandic cortex and basal ganglia Asymmetric rigidity Dystonia Myoclonus and apraxia of one limb Alien limb ```

Corticobasal syndrome

Amount of acetylcholine released per impulse declines on repeated activity

Presynaptic rundown

Most common cause of acute gen weakness leading to ICU admission

GBS AND MG

Parkinson plus syndrome with marked DYSAUTONOMIA

Shy Drager Syndrome

Tremor Bradykinesia Cervical oromandibular With DYT-3 mutation

X linked Dystonia-Parkinsonism

Drugs avoided in MG

Gentamycin Ciprofloxacin Succinylcholine Except insulin

Neuropathologic feature of PD

Lewy body