NEURO-DEV Flashcards
Drug of choice: ABSENCE SEIZURE
Ethosuximade or Valproate
2nd line: Clonazepam, Clobazam, Acetazolamide
peak is 4-10yo
mostly females
Drug of choice: JUVENILE MYOCLONIC SEIZURE
Valproate
2nd line: Levetiracetam,
LIFESTYLE CHANGES - Regulate sleep-wake cycle
Most common: Idiopathic childhood focal epilepsy
ROLANDIC EPILEPSY
- Focal epilepsy
- self-limited
- Previously called BECTS
- centro-temporal spikes
TRIAD of Dandy Walker Syndrome
- Cystic enlargement of 4th ventricle
- Hypoplasia of the cerebellum
- Enlargement of the posterior fossa
Myelomeningocele + Hydrocephalus =
Chiari Type II
4 obligatory criteria for MUSCULAR DYSTROPHIES
- A Primary myopathy
- Has a Genetic basis
- Course is progressive
- Degeneration and death of muscle fibers occur in some stage in the disease
DOC Rolandic Epilepsy
Carbamazepine or VPA
peak is 7-10yo
mostly males
Characteristic imaging for MESIO-TEMPORAL LOBE WITH HIPPOCAMPAL SCLEROSIS (mTLE)
MRI: layer of atrophy in the Hippocampus
> FOCAL seizures
Epilepsy occuring at sleep (near sleep onset or near awakening)
Rolandic Epilepsy
(Ayaw matulog ni Roland)
Treatment for mTLE
Na channel blockers (Carbamazepine) or Levetiracetam (SV2A blockers)
Brachial plexus defect: Waiter’s tip deformity nerve affected
C5, 6
> Erb palsy
Characteristic finding on EEG for West Syndrome
Hypsarrhythmia
> Diffue high voltage slow activity associated with diffuse multifocal spikes in wakefulness
Major risk factors for Febrile Seizures recurrence
- Young age at onset <1yr
- Duration of fever <24hrs
- Fever 38-29degC
Most severe form of epileptic encephalopathies
Dravet Syndrome
- Severe myoclonic epilepsy in infancy
- Males > Females
- onset is 6-15mos
- Presentatiopn: Febrile seizures
Triggers of Dravet Syndrome
- Fever
- Fatigue
- Photophobia
- Pattern sensitivity
- Exercise
- Excitement
Normal Flow of CSF
Come Let’s Migrate To States For Money Love Sex Ahh
Choroid Plexus > Lateral ventricle > foramen Monroe > Third ventricle > aqueduct of Sylvius > Foramen of Magendie > Luschka > Arachnoid villi
Most prominent sign of Dravet syndrome
Hypotonia (esp in 1st yr of life)
Ophthalmic findings in Aicardi syndrome
Chorioretinal lacuna in fundoscopy
Mobius syndrome: Agenesis of CN __
CN VII
Side effect of Valproic Acid
Hepatotoxic
Teratogenic
Weight gain
Klumpke palsy
C8-T1
> poor grip
> intrinsic hand muscles are affected
> claw hand
Spinal Muscular Atrophy vs. Muscular Dystrophy
SMA:
> CK levels are normal or slightly elevated
> EMV: neuropathic changes
Guillain Barre Syndrome
> Other name
> Features (3)
Acute Inflamnatory Demyelinating Polyradiculopathy
- Motor weakness:
- ASCENDING
- SYMMETRIC
- DISTAL > PRXIMAL - Areflexia
- Sensory symptoms: pain, paresthesia
Most common cause of unilateral/bilateral ptosis
Myasthenia Gravis
Hydrocephalus vs hydrancephaly
Hydrocephalus - accumulation of CSF
Hydrancephaly- absent cerebral hemispheres
MC form of craniosynostosis
Scaphocelphaly
> Premature closure of sagittal sutures
> long and narrow skull
> M>F
Frontal plagiocephaly
> 2nd MC
> unilateral flattening of the forehead
> F>M
Hallmark of neurofibromatosis
Cafe au lait macules (CALMs)
Hypomelanotic macules and elevated, rough plaques of skin with a predilection for the lumbar and gluteal regions that develop in late childhood or early adolescence are seen in what neurocutaneous disease?
A. Multiple sclerosis
B. Tuberous sclerosis
C. Neurofibromatosis
D. Sturge Weber Syndrome
B. Tuberous sclerosis
Ash leaf spots
> hypomelanotic macules
Shagreen patches
> elevated, rough plaques of skin with a predilection for the lumbar and gluteal regions that develop in late childhood or early adolescence
Neurocutaneous disease w the ff features: port-wine stain, seizures, angiomas.
A. Multiple sclerosis
B. Tuberous sclerosis
C. Neurofibromatosis
D. Sturge Weber Syndrome
D. Sturge Weber Syndrome
NF1 vs NF2
No CALMs, seizures and axillary freckling in NF2
NF2: schwannomas
Oscillating, rhythmic movements about a fixed point, axis, or plane
A. tremor
B. Tic
C. Chorea
D. Dystonia
E. Myoclonus
F. Athetosis
A. tremor
Involuntary, sudden, rapid, abrupt, repetitive, nonrhythmic, simple or complex motor movements or
vocalizations (phonic productions).
A. tremor
B. Tic
C. Chorea
D. Dystonia
E. Myoclonus
F. Athetosis
B. Tic
Involuntary, continual, irregular movements or movement fragments with variable rate and direction that
occur unpredictably and randomly.
A. tremor
B. Tic
C. Chorea
D. Dystonia
E. Myoclonus
F. Athetosis
C. Chorea
Slow, writhing, continuous, involuntary movements
A. tremor
B. Tic
C. Chorea
D. Dystonia
E. Myoclonus
F. Athetosis
F. Athetosis
MC degenerative ataxia
Ataxia telangiectasia
> autosomal recessive
starts 2yo
loss of ambulation by adol
Most severe form of CP
Spastic quadriplegia
MC neuropathologic finding on MRI in children w spastic diplegia
A. Periventricular leukomalacia
B. Multicystic encephalomalacia
C. Cortical malformation
Periventricular leukomalacia
The hallmark of a neurodegenerative disease is __.
regression and progressive deterioration
of neurologic function with loss of speech, vision, hearing, or locomotion, often associated with seizures, feeding difficulties, and impairment of intellect.
