NEURO-DEV Flashcards by Maria Cristina Mariano

Drug of choice: ABSENCE SEIZURE

Ethosuximade or Valproate
2nd line: Clonazepam, Clobazam, Acetazolamide

peak is 4-10yo
mostly females

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Drug of choice: JUVENILE MYOCLONIC SEIZURE

Valproate
2nd line: Levetiracetam,
LIFESTYLE CHANGES - Regulate sleep-wake cycle

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Most common: Idiopathic childhood focal epilepsy

ROLANDIC EPILEPSY
- Focal epilepsy
- self-limited
- Previously called BECTS
- centro-temporal spikes

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TRIAD of Dandy Walker Syndrome

Cystic enlargement of 4th ventricle
Hypoplasia of the cerebellum
Enlargement of the posterior fossa

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Myelomeningocele + Hydrocephalus =

Chiari Type II

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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

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DOC Rolandic Epilepsy

Carbamazepine or VPA
peak is 7-10yo
mostly males

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Characteristic imaging for MESIO-TEMPORAL LOBE WITH HIPPOCAMPAL SCLEROSIS (mTLE)

MRI: layer of atrophy in the Hippocampus
> FOCAL seizures

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Epilepsy occuring at sleep (near sleep onset or near awakening)

Rolandic Epilepsy

(Ayaw matulog ni Roland)

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Treatment for mTLE

Na channel blockers (Carbamazepine) or Levetiracetam (SV2A blockers)

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Brachial plexus defect: Waiter’s tip deformity nerve affected

C5, 6
> Erb palsy

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Characteristic finding on EEG for West Syndrome

Hypsarrhythmia
> Diffue high voltage slow activity associated with diffuse multifocal spikes in wakefulness

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Major risk factors for Febrile Seizures recurrence

Young age at onset <1yr
Duration of fever <24hrs
Fever 38-29degC

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Most severe form of epileptic encephalopathies

Dravet Syndrome
- Severe myoclonic epilepsy in infancy
- Males > Females
- onset is 6-15mos
- Presentatiopn: Febrile seizures

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Triggers of Dravet Syndrome

Fever
Fatigue
Photophobia
Pattern sensitivity
Exercise
Excitement

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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.