Neurology Flashcards by Vanessa Soetanto

Tourette Syndrome definition

motor AND vocal tics
> 1 year

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What are the red flags for headaches and what is the next step if they are present?

Acute/subacute
Progressive
Posterior
Pressure
Early morning/awakening
Constant
Emesis > nausea
Diplopia
Provoked by pressure, papilledema, pregnancy
Younger <6yo

MRI, LP if you’re thinking pseudomotor cerebrii

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Benign rolandic epilepsy

Biphasic independently bilateral centrotemporal spikes, near central culcus (in light sleep)
Focal motor epilepsy
5yo - 10 yo
Infrequent brief focal motor seizures – in sleep
Arm and facial twitching, speech arrest, oropharyngeal guttural sounds, hypersalivation, preservation of consciousness

+/- GTC

No need to treat but if you want, oxcarbazepine and keppra
Prognosis: remit by 16 yo

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3 Hz spine and slow wave

Absence
Ethosuximide –> valproic acid –> lamotrigine

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Triad of juvenile myoclonic epilepsy and age of onset

myoclonic seizures in early morning
GTCs
Abscence

late school-age & adolescent
Precipietated by alcohol, stress, sleep deprivation

Prognosis: persists into adulthood

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Panayiotopoulos syndrome

Infrequent autonomic seizures
1-4 yo
Ictal emesis, mydriasis, pallor, cyanosis
Out of sleep
Preservation of consciousness
–> status epilepticus or hemiclonic/clonic movements
Frequently Mistaken for encephalitis

Prognosis: grow out of it after few years

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Sx of Chiari II malformation

swallowing difficulties, stridor, hoarse voice, disordered breathing, choking, breath0holding spells

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What is myelomeningocele

incomplete closure of posterior neural tube
Exposed meningeal sac
Results in hydrocephalus and Chiari II

Associated with agenesis of corpus callosum, polymicrogyria, heterotopia, structural micro-anomalies

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Sx of hydrocephalus

increased head circ
bulging fontanelle
Restricted upward gaze (sun-setting eyes)
VI nerve palsies (inability to abduct eyes)
Lethargy

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Rapidly progressing scending paralysis after hiking, ddx and treatment

Tic paralysis

Rocky Mountain Spotted Fever Dermacentor andersoni or dermacentor variabilis

Tx: removal of tic (improvement in 1h to few days)

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Transverse myelitis sx

both MOTOR and SENSORY deficit, ofter bowel and bladder dysfunction

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Dandy Walker malformation

congenital malformation of 4th ventricle and cerebellum

delayed walking/crawling
hydrocephalus

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Someone comes in lethargic, found to have brain abscesses on MRI, what is the next step (per PREP)

blood cultures
prior to administering IV abx and calling neurosurgery

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If a child has painful torticollis, not improving, cant move head past midline, what are you suspicious for? What imaging should you get

Atlantoaxial rotary subluxation (C1-C2)

CT cervical spine
Tx: conservative, resolve in 1-2 weeks

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Febrile seizure 5 criteria

OOGLO

Occur in child 6mo-5yo
Occur in developmentally normal child
Generalized
Last <5 min
Once in a 24-hour period

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Risk of devepoling epilepsy after febrile seizure

slightly higher

Recurrence:
1/3 of children with simple febrile seizure
More likely if first seizure <12mo

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Diagnosis of migraine

> 4 lifetime attacks lasting 2-72 hours
2 of 4: photophobia, phonophobia, nausea, vomiting
+/- aura

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Tx of migraine (stepwise)

lifestyle migraine management
abortive medications and plan
Preventive medications

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Acute flaccid myelitis

acute paralysis and areflexia of 1 or more limbs
preserved sensation
normal mental status
Onset: within 1 week of viral symptoms
MRI: central inflammation
LP: lymphocytic pleocytosis
Tx: supportive

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Acute disseminated encephalomyelitis (ADEM)

Post-infectious
Needs encephalopathy
Seizure, headache, ataxia, paralysis, paresthesias
Brain MRI: demyelination, focal/multifocal lesions in white matter without prior lesions
Tx: steroids

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Guillain Barre tx

IVIG
plasmapharesis

Idiopathic transverse myelitis MRI findings and tx

longitudinal lesion, multiple segments involving white and gray matter

Tx: steroids

Juvenile myoclonic epilepsy EEG findins and Tx

polyspike and wave at 4-6Hz frequency
Tx: valproic acid (not in adolecent girls –> teratogenic), zonisamide, topiramate, keppra

Which AED exacerbates myoclonus in some children

lamotrigine

Progressive myoclonic epilepsy

myoclonic GTC progressive neurodegeneration Cognitive decline, ataxia, neuropathy, myopathy

When does postconcussive syndrome start?

4 weeks after concussion, lasts 3 mo

Risk factors for developing post-concussive syndrome

Pre-existing psych disorders Multiple prior concussions History of migraine HAs Learning disorder Severe initial symptom, LOC for >1 min, vestibular symptoms

Defining features of Rett

only GIRLS Normal development, then REGRESSION with loss of speech, stereotyped hand motion, and gait abnormalities Acquired microcephaly (was well then falls off curve) MECP2 on X chromosome

X-linked adrenoleukodystrophy

genetic peroxisomal disorder affecting CNS myelin (no oxydation of very long chain fatty acids BOYS between 4-8 Sx: ADHD, vision complaints, global developmental regression + adrenocortical dysfunction

MRI of x-linked drenoleukodystrophy

bilateral posterior-predominant inflammatory demyelination affecting parieto-occipital lobes

Tx of X-linked adrenoleukodystrophy

HSCT if early enough

Landau-Kleffner Syndrome

Acquired epileptiform aphasia Early childhood language regression 2/2 freq epileptiform discharges in parieto-occipital regions Activated by sleep

Acute transverse myelitis is associated with?

neuromyelitis optica MS

Diagnosis and tx of duchenne muscular dystrophy

CK corticosteroids

Diagnosis of narcolepsy

4 clinical features for at least 3 mo Excessive daytime sleepiness Cataplexy Hypnagogic hallucinations Sleep paralysis

What is myasthenia gravis associated with and what imaging should be done?

Thymoma or thymic hyperplasia Get a Chest CT

Treatment of juvenile myasthenia gravis

thymectomy pyridostigmine (acetylcholinesterase inhibitor prevents degradation of acetylcholine in NM junction) Immunomodulation to decrease Ab (steroids, cellcept, azathioprine)

Management of myasthenia gravis exacerbations

IVIG Plasma exchange

What do you use metaiodobenzylguanidine (MIBG) scans to evaluate?

opsoclonus-myoclonus-ataxia paraneoplastic syndrome to evaluate for neuroblastoma

In Anti-NMDA encephalitis, what further imaging should you obtain?

CTAP to look for occult tumors

What is the Miller Fisher variant of guillan-barre syndrome?

triad of ophthalmoplegia, areflexia, ataxia Autoantibodies to ganglioside GQ1b, GT1a and GD3

What is albuminocytologic dissociation

high protein, normal cell count in CSF supportive of acute inflammatory demyelinating polyneuropathy (AIDP)

Signs and sx of acute cerebellar ataxia

HEAD BOBBING (titubation), dysarthria, dysmetria Prior infection Affects 2-5 years Classically 2/2 varicella infection, but can be others

Presentation of SMA

Regression of milestones Muscle weakness, hypotonia, diminished reflexes Pathophys: anterior horn cell dysfunction and loss Legs>arms

Definition of status epilepticus

continuous seizure activity or clustering of seizures without return to baseline for longer than 30 min early SE: sz lasting between 5-30 m Refractory: continuous despite treatment with 2-3 AED loads

Closed-lip schizencephaly

congenital disorder of cortical migration

Spastic diplegia, where is the predominant site of spasticity and most common cause?

legs Premature (periventricular leukomalacia), prenatal/postnatal infections, brain malformations, anoxic injury

Extrapyramidal/dyskinetic CP, what does it effect and what are symptoms?

arms > legs bradykinesia, choreoathetosis, hemiballismus, dystonia Causes: hypoxic-ischemic encephalopathy and kernicterus

What are fifth-day fits and how do you treat it

benign neonatal seizure tx: AED familial, should go away within few days to week after birth

Most common cause of neonatal seizure

hypoxic ischemic encephalopathy

Infantile epileptic encephalopathies EEG pattern

burst suppression pattern on EEG treatment-resistant

Pyridoxine dependent epilepsy tx

not AED pyridoxine +/- folinic acid