Neurology Part 2 Flashcards
transient occurence of signs or symptoms due to abnormal excessive or synchronous neuronal activity
Seizure
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
ILAE commission on classification and terminology
Clinical feature
EEG
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.
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
New classification emphasizes on
Awareness (intact or impaired)
Nature of onset (motor or nonmotor)
Focal with intact awareness may be
Motor (tonic, clonic, myo)
Nonmotor (sensory, autonomic, emotional) without awareness impairment
Spread of activity over a progressively larger region of motor cortex
Jacksonian march - focal motor
Localized paresis for minutes to many hours in involved region following seizure
Todd’s paralysis - focal motor
Seizure continuing for hours or days
Epilepsia partialis continua - focal motor
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
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
During start of ictal phase, the patient is in
motionless stare marking onset of impaired awareness
Focal seizures with impaired awareness also may come with
automatism (involuntary automatic behavior)
chewing
lip smacking
picking movement of hand
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
Absence symptoms
Rapid blinking of eyelid
Chewing
Small amplitude clonic movement of hands
Childhood 4-10 years or early adolescence
First clue to absence is unexplained
daydreaming and decline in school performance
Absence electrophysiological hallmark
Provoked by
generalized, symmetric, 3 Hz spike and slow wave discharge beginning and ending suddenly superimposed on normal EEG background
Hyperventilation
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
Tonic contraction of muscle of expiration and larynx at onset will produce a loud moan
ictal cry
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
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
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
Pathologic myoclonus seen in
EEG shows
Metabolic d/o
Degenerative CNS
Anoxic brain
Bilaterally synchronous spike and slow wave
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