Neurology Flashcards by Samia Aleem

Most common cause of CP in term neonates in the US?

HIE

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Most common neurologic complication of prematurity?

IVH

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Most sensitive time for MRI for hypoxic ischemic brain injury?

24-96 hours

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MRI at what time shows full extent of brain injury (HIE)?

10 days (between 7-21 days)

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Gold standard for diagnosing seizures?

Continuous EEG

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Are all neonatal seizures correlated clinically?

No, 80-90% of seizures have NO clinical correlation

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When does edema peak in HUS for HIE?

24 hours

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When can you see diffusion changes on MRI in HIE?

Present 7-10 days, then pseudonormalize

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What is basal ganglia injury predictive of?

Severity of motor injury (Cerebral palsy)

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What is posterior limb, internal capsule injury predictive of?

Ability to walk at 2 years

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Most common type of neonatal stroke?

Arterial ischemic stroke

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Most common presenting sign of stroke?

Seizures (>75%)

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Name factors that decrease cerebral blood flow

Hypocarbia, hypotension, hypothermia and polycythemia decrease cerebral blood flow

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An abnormality at which stage leads to anencephaly, and failure of what causes it?

Failure of the anterior tube closure during primary neurulation within first 26 days (weeks 3-4)

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Maternal risk factors for anencephaly

Maternal hyperthermia, copper, zinc and folate deficiencies

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Prenatal diagnostic factors for anencephaly

Elevated alpha fetoprotein
Polyhydramnios
Other anomalies: CDH, CHD, Trisomy 13 and 18, omphalocele
Detected on ultrasound at week 14-15

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What abnormality leads to encephalocele and when does it occur?

Failure of closure of rostral tube during primary neurulation (weeks 3-4)

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Most common location for encephalocele

Occipital

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What is the prognosis of an encephalocele depend on?

The amount of brain tissue in the sac

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Why do myelomeningoceles happen? (defect)

Failure of posterior neural tube

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In which location are most meningomyeloceles?

Lumbar

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What does prognosis of a myelomeningocele depend on?

Level of lesion (lower the lesion, better the outcome), also presence of other anomalies

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What is an Arnold Chiari Type 1 malformation associated with?

Syringomyelia, hydromelia, skeletal anomalies (scolisos most common)

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What is an Arnold Chiari Type 2 malformation associated with?

Myelomeningocele and hydrocephalus

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Familial features associated with holoprosencephaly

Single maxillary central incisor Ocular hypertelorism Midfacial abnormalities

Aprosencephaly occurs due to a defect in?

Formation during the prosencephalic stage

Holoprosencephaly occurs due to a defect in?

Cleavage during prosencephalic stage

What changes in paCO2 and paO2 lead to increased cerebral blood flow?

Increase in paCO2 and decrease in paO2 cause increased CBF

Formula to calculate cerebral perfusion pressure

Mean arterial pressure - intracranial cerebral pressure

Changes in hemoglobin concentration and proportion of fetal hemoglobin leading to increased cerebral blood flow?

Decreased hemoglobin concentration | Increased proportion of fetal hemoglobin

Most common type of craniosynostosis? Caused by closure of which suture?

Dolicocephaly or scaphocephaly | Closure of saggital suture

Craniosynostosis that is associated with Crouzon and Apert syndrome

Frontal plagiocephaly | Unilateral closure of coronal suture

Craniosynostosis associated with Carpenter syndrome

Brachycephaly | Closure of bilateral coronal sutures

At what age does the anterior fontanel completely close by

Age 2 years

Syndrome with bilateral facial paresis?

Mobius syndrome | Secondary to hypoplasia or absence of cranial nerve nuclei

Cause of hypertonia (injury to which structure)

Corticospinal tract or extrapyramidal system

Persistence of palmar grasp is characteristic of

athetoid cerebral palsy | Normally disappears by 2-4 months

Where is the choroid plexus located

Atrium of lateral ventricles

When do choroid plexus cysts appear and disappear by? What syndrome can they be associated with?

Appear by week 11, disappear by week 26. Can be associated with trisomy 18

Major cation in CSF?

Sodium

At which gestational age do delta brushes appear and disappear on EEG?

Appear at weeks 29-30 | Disappear at week 36-37 from central region and weeks 38-40 from occipital region

What is hypsarrhythmia seen with?

Infantile myoclonic spasms

What are central positive sharp waves seen with?

Premature infants with PVL

Most common pattern of cerebral injury seen with term HIE

Selective neuronal necrosis

Which artery is the most common site for focal cerebral ischemia

Left MCA. Left hemisphere is most commonly affected

Most common clinical sequela of PVL?

Spastic diplegia (lower extremities more affected than upper extremities)

Most common location of neuronal injury in hypoglycemia?

Bilateral, posterior occipital cortex

Which nerve roots are damaged in Erb-Duchenne

C5-C7

Which nerve roots are damaged in Klumpke's

C8-T1

Where is the defect/pathogenesis in SMA type 1?

Lower motor neuron - degeneration of anterior horn cell

Inheritance pattern of SMA type 1

Autosomal recessive, chromosome 5

EMG features in neonatal myasthenia gravis

Progressive decline in amplitude with repetitive nerve stimulation, returns to baseline after a period of rest or administration of neostigmine

Inheritance pattern for myotonic dystrophy

Autosomal dominant, chromosome 19. Expanded repetition of CTG Inherited almost entirely from mother

What determines the severity of congenital myotonic dystrophy

Number of CTG repeats

Muscle biopsy in congenital myotonic dystrophy

Abnormal: small and round muscle fibers, large nuclei, sparse myofibrils

EMG in congenital myotonic dystrophy

Myotonic changes eliciting a dive bomber sound

Prader Willi genetics

Deletion of 15q 11q13, some caused by maternal uniparental disomy Deleted piece always of paternal origin

Prader Willi clinical triad

Hypotonia, cryptorchidism, poor feeding

Weight and growth pattern in Prader Willi

Failure to thrive during infancy, followed by obesity that presents between 6 months to 6 years

Most common location for intracranial tumor

Supratentorial

Most common presenting sign for an intracranial tumor

Increasing head circumference with bulging fontanel

Features of Sturge Weber

Port wine stain, facial hemangioma (distribution of 1st division of trigeminal nerve). Present at birth Glaucoma CNS ipsilateral tramline intracortical calcifications Seizures Hemiparesis

Features of tuberous sclerosis

``` Autosomal dominant Hypopigmented ash leaf macules, greatest on trunk or buttocks Cardiac rhabdomyomas CNS tumors Enamel pits in teeth Seizures ```

Features of neurofibromatosis

Autosomal dominant, chromosome 17 Cafe au lait spots, dont cross midline, have sharp borders, multiple Freckling Associated tumors: cutaneous neurofibromas, schwannoma, pheochromocytoma Seizures

Features of McCune Albright

``` Irregular brown pigmentation Fibrous dysplasia of bones Precocious puberty Hyperthyroid Hyperparathyroid Pituitarty adenomas ```

Features of von Hippel Lindau

``` Autosomal dominant, chromosome 3 Increased tumor growth CNS tumor (hemangioblastoma, most commonly in cerebellum) Multiple systemic hemangiomata Retinal angiomas Pheochromocytomas ```

Most common type of CP with extreme prematurity

Spastic diplegia

Most common type of CP with bilirubin encephalopathy

Athetoid CP

Most common cause of multiple congenital contractures?

Amyoplasia

Cause of benign familial neonatal seizures

Potassium channel | Positive family hx

Gold standard for diagnosing neonatal seizures

Continuous EEG

Normal EEG pattern for premies?

Discontinuous, switches to continuous ~31 weeks Delta brushes start disappearing ~36 weeks

Persistence of which reflex after 2-4 months is characteristic of athetoid CP?

Palmar grasp | Appears 28 weeks, well established 32 weeks, disappears by 2-4 months

What is an asymmetric Moro suggestive of?

Peripheral nerve injury

Persistence of tonic neck reflex after 6 months is suggestive of?

Focal cerebral abnormalities

Falx laceration that leads to subdural hemorrhage is a result of rupture of which vessel?

Inferior saggital sinus

Most common birth injury (excluding head stuff)?

Clavicle fracture

Horner's syndrome is a result of injury to which nerve root?

Most common traumatic nerve injury?

Facial nerve injury

How do you bypass flexor predominance in the neonatal period during an examination?

By assessing both pronation and supination

Signs of upper motor neuron dysfunction?

Hyperreflexia

HIE cooling temperature?

33.5°C to 34.5°C

Which anti-epileptic is most teratogenic?

Valproate

Which anti-epileptic has the most effect on cognition?

Valproate