Neuro Flashcards by Misun Jung

What decreases CBF (cerebral blood flow)?

Decreased PCO2
Increase paO2
Increase Hb [ ]
Decreased fetal Hb

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What are U/S findings with Meningomyelocele?

Lemon 🍋 sign - compression due to CSF leak
Banana 🍌 sign - compressed cerebellum

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Mother with prolonged labor
Polyhydramnios
Baby with respiratory failure and tent shaped mouth, and hypotonia
Diagnosis? Genetics? Cause? Maternal symptoms?

Myotonic Dystrophy
AD; chromosome 19 (DMPK gene)
Caused by CTG repeats- severity determined by # of repeats–> disorder of muscle, Dysfunctional Na /K channel

Polyhydramnios 2/2 difficulties swallowing
Maternal: Prolonged labor bc uterine dysfunction in mom, inabilty to open eyes, delayed release hand grip

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Baby with decreased fetal movements, hypotonia, bell shaped chest on CXR, frog leg position

Dx?
Test?

SMA

EMG - Abnormal activity with fibrillation and fasiculations

Bulbar weakness (poor suck/swallow, weak cry, tongue fasiculations)
Marked head lag
Facial sparing
Absent deep tendon réflexes (aréflexia)
Normal diaphragmatic function

AR, chromosome 5

Degeneration of anterior horn cell

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Earliest imaging modality to detect HIE

MR spectroscopy
Can detect inc in brain lactate concentrations as soon as 2-8 hrs after injury

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Neural tube defects have highest rate among which population?

Hispanic patients

Teenage

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Which is most effective intervention to decrease risk of IVH?

Prenatal steroids

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Neonates with transient neonatal myesthenia gravis present most commonly with?

Symptomatic within 72 hours: Poor suck/swallow, weak cry, resp distress and ptosis/ophthalmoparesis

Atypical presentation can be arthogryposis multiplex congenita

Majority need neostigmine
Resolution of symptoms in 2 months

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Cerebral tissue hypoxia results in reduction of?

ATP and Phosphocreatinine->influx of intracellular calcium

Protease activation leads to cytoskeletal disruption

Protease and phospholipase activation leads to apoptotic Pathways

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Cellular mechanism of HIE

Cerebral tissue hypoxia –> decreased production of ATP and phosphocreatine –> cell membrane depolarization/ Ca influx

Ca–>
Endonuclease activation that results in nuclear injury
Protease activation leading to cytoskeleton disruption
Protease and phospholipase activation that results in free radical production and activation of the apoptotic pathways
Nitric oxide synthase activation
An increase in excitatory neurotransmitter (glutamate) release

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Most common risk factors for perinatal brachial plexus palsy?

Shoulder dystocia (45%)

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Describe DANDY walker malformation

Agenesis of cerebellar vermis, cystic dilation of 4th ventricle and enlargement of posterior fossa

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Describe Arnold chiari malformation type 1

Present in childhood/adulthood
Displacement of cerebellar tonsils below foramen magna
4th ventricle and brainstem normal
Syringo or hydromelia
Ass w scoliosis

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What syndromes present w agenesis of corpus callosum?

Dandy walker, holoprosencephaly
Non ketotic hyperglycinemia, pyruvate dehydrogenase deficiency
T8, 13,18
Aicardi syndrome-infantile spasm, Coloboma hypotonia
Zellweger-peroxisomal disorder

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A GMFCS of 5 indicates a higher or lower likelihood of severe limitations in CP?

Higher (5)
lower (1)

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MC Type of CP in bilirubin encephalopathy

Athetoid (increased activity with reduced tone)

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Most common CP in preterm neonates is ….

Spastic diplegia

Cognitive function is not usually affected (unless quadriplegia)

Fine motor usually not affected

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Prenatal repair of myelomeningocele is associated with

Risk of sx: maternal morbidity, preterm birth, increased Uro complications

Stopped early because of a reduced need for CSF shunt in prenatal intervention group by 12 months

Decrease in fetal/neonatal death

Improved mental/motor function at 30 months

Sx recommended in: Singleton pregnancy, Lesion at spinal level T1-S1, normal karyotype and Arnold Chiari type 2

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What is the mechanism of action of hypothermia In decreasing hypoxic injury?

Decrease intracellular Ca influx and mitochondrial dysfunction
Decrease free radical production
Decrease in metabolic demands

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What are long term outcomes for SSRI- exposed children ?

ADHD

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Term infant
Ashkenazi Jewish family Hypotonia
Autonomomic issues (loose, irritability, Pale blotchy skin)

(think NAS with hypotonia)

Riley - Day syndrome (Familial dysautonomia)

AR
Chromosome 9

Dx- pupil construction in response to metacholine eye drops

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Describe CN and function

I- Olfaction
II- Vision, optic fundi
III- Pupils
III, IV, VI-Extraocular movements
V-Facial sensation, masticatory power
V1: ophthalmic
V2:maxillary
V3: mandibular
VII- Facial movements
VIII-Auditory
V, VI, IX, X, XII-Sucking and swallowing
XI-Sternocleidomastoid function
XII-Tongue
VIII, IX- Taste

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Pt with expressionless face. What’s the syndrome?

Mobeus syndrome

Secondary to hypoplasia or absence of CN nuclei

No treatment or recovery

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Which EEG pattern is seen in PVL?

Central positive sharp waves

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What is the diagnostic test for neonatal myesthenia gravis?

Anti-acetylcholine receptor antibody assay Anti-AChr 85% (nicotinic receptor) Anti-Muskr 15% (muscle specific receptor tyrosine kinase)

Which is the primary cellular target in PVL?

Oligodendrocyte progenitor cell Pre-Oligodendrocyte most abundant during peak incidence of PVL

Brachial plexus injury associated with Horners

Klumpke (T1)

When do u expect full recovery of brachial plexus injuries?

6 months @2 wk you see improvement If residual impairment at 15 months, usually persists

Brachial plexus injury with Phrenic nerve paralysis

Erb (C4-C5)

Term female with focal clonic seizure at 5 days of life confirmed on EEG. No fhx of seizure. What type of seizure is she exhibiting?

Benign neonatal seizure aka “fifth day fits”

The location of white matter injury in preterm neonates is specific to which area? And why?

Periventricular area Distribution of oligodendrocyte progenitors

What is the most common congenital posterior fossa malformation?

Dandy Walker Malformation 1. Agenesis or hypoplasia of the cerebellar vermis 2. Cystic dilation of the 4th ventricle 3. Enlargement of the posterior fossa * Associated with hydrocephalus * Increased risk of motor and cognitive delays

Most common hemisphere to be affected with term focal or multi focal ischemia? Which artery MC involved?

Left hemisphere Left MCA

How does Periventricular hemorrhagic infarction occur?

Hemorrhagic necrosis of PV white matter related to IVH. IVH obstructs blood flow in terminal vein leading to venous infarction. Peak ~4 days of life

Risk factors predispose premies to PVL

1. Periventricular vascular anatomic factors 2. Cerebral perfusion pressure depends on systemic BP 3. Increased vulnerability periventricular glial cells 4. Insult leading to oligodendroglia cell death and myelin deficiency

Pattern of hypoglycemic injury on MRI?

Related to duration rather than severity of hypoglycemia Involves bilateral posterior occipital cortex. Middle cerebral infarction and basal ganglia/thalamus abnormalities

The effect of hypoglycemia on MRI

Corticospinal tract injury - basal ganglia and thalamus abnormalities - not how low - import how long (duration)?

Neonates with congenital myotonic dystrophy at highest risk for?

GI dysfunction Resp dysfunction/hypotonia Endocrinopathies-hypothyroidism and hyperparathyroid Adults risk for fatal arrhythmias

MCC of CP in term neonates

HIE (1-2/1000 term births)

Which is the most common presentation of Arterial ischemic stroke?

Nonspecific Presentation, Seizures most common presenting sign (>75%) ~20% of neonates with seizure have stroke * Lethargy * Vital sign fluctuation * Poor feeding * NOT hemiplegia Multiple risk factors in setting of transient hypercoagulability of newborn

What are the proposed mechanisms for IVH?

Fragile vessels in germinal matrix Clinical instability Fluctuating CBF -Increases CBF with Impaired auto regulation (bolus, hypercarbia, anemia/hypoglycemia) -Decrease CBF from hypotension

How does Grade IV IVH develop?

GM hemorrhage leads to periventricular venous congestion. This leads to PV ischemia which can lead to periventricular infarction

Complication of endoscopic third ventriculostomy with choroid plexus ablation in infant with hydrocephalus?

Diabetes insipidus Likely related to hypothalamus injury Tx: water replacement, ADH

B/l vocal cord paralysis is associated with which conditon?

Arnold Chiari II

Cranial nerves involved in gag reflex

9 and 10

Describe anomalies in goldenhar syndrome

Oculoauriculo vertebral syndrome Ear Tag on tragus

Name associated findings with Meckel Gruber syndrome?

Occipital encephalocele Polydactyly Cystic dysplastic kidneys Leads to ciliary dysfunction AR

Arnold chiari 2

Elongation and caudal displacement of cerebellar tonsils, 4th ventricle choroid plexus Ass w/Hydrocephalus

Arnold chiari what kind of defect

Primary neurulation defect

What is scaphocephaly/dolichocephaly characterized by?

MC craniosynostosis, M>F Premature closure of the sagittal suture

What are the characteristics of frontal plagiocephaly?

Premature closure of unilateral coronal suture High incidence of developmental abnormalities and mental deficiency, Associated with Crouzon and Apert syndrome Early correction to prevent neurological sequelae

What is brachycephaly characterized by?

* Premature closure of bilateral coronal sutures * Can be observed in Carpenter syndrome

What is trigonocephaly characterized by?

Premature closure of metopic suture Associated with hypotelorism

What is **occipital plagiocephaly** characterized by?

Premature closure of the unilateral lambdoid suture Increased risk for increased intracranial pressure Increased risk for mental deficiency Surgery as soon as possible

Facial nerve palsy

Unilateral L>R Recovers in 1-3 weeks

Development of Nervous System- name 6 processes in order

Primary/Secondary neurulation Prosencephalic Development Neuronal proliferation Neuronal Migration Neuronal Organization Myelination

Primary/Secondary Neurulation disorders

Primary Neurulation: brain and spinal cord Anencephaly Encephalocele MM Arnold Chiari Malformation Secondary neurulation: lower spinal cord tethered cord Teratoma Spinal cysts

Prosencephalic Development

Anterior end of brain and midline development Aprosencephaly Holoprosencephaly Agenesis of corpus callosum Absence of septum pallucidum Septo Optic Dysplasia

Neuronal Proliferation

Micrencephaly Macrencephaly

Neuronal Migration

Lissencephaly Schizencephaly Pachygyri Polymicrogyria

Neuronal organization

T21 Fragile X Angelman Prematurity Autism

Tuberous Sclerosis- genetics, clinical features

AD, chromosome 9/16 Ash leaf macules (butt/Trunk) Cardiac rhabdomyoma CNS tumors Seizures

Most common cause of Seizures

1. HIE 2. Stroke 3. IVH 4. Cerebral malformations

Grasp reflex Established by, disappears by

32 weeks 3 months

Moro Established by, disappears by

38 weeks 4 months

Tonic Neck Established by, disappears by

2 months 6 months

Stepping Established by, disappears by

Term 2-3 months

Strongest risk factors for hearing loss

HIE Craniofacial abnormalities Syndrome w/ Hearing loss

Facial palsy recovery

1-3 weeks

Abnormal hearing screen

>35 dB

Neuro Flashcards

(71 cards)