Neurology Flashcards
Tourette Syndrome definition
motor AND vocal tics
> 1 year
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
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
3 Hz spine and slow wave
Absence
Ethosuximide –> valproic acid –> lamotrigine
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
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
Sx of Chiari II malformation
swallowing difficulties, stridor, hoarse voice, disordered breathing, choking, breath0holding spells
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
Sx of hydrocephalus
increased head circ
bulging fontanelle
Restricted upward gaze (sun-setting eyes)
VI nerve palsies (inability to abduct eyes)
Lethargy
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)
Transverse myelitis sx
both MOTOR and SENSORY deficit, ofter bowel and bladder dysfunction
Dandy Walker malformation
congenital malformation of 4th ventricle and cerebellum
delayed walking/crawling
hydrocephalus
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
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
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
Risk of devepoling epilepsy after febrile seizure
slightly higher
Recurrence:
1/3 of children with simple febrile seizure
More likely if first seizure <12mo
Diagnosis of migraine
> 4 lifetime attacks lasting 2-72 hours
2 of 4: photophobia, phonophobia, nausea, vomiting
+/- aura
Tx of migraine (stepwise)
- lifestyle migraine management
- abortive medications and plan
- Preventive medications
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
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
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
