Neurology

Question 1

Neuro assessment

Answer 1

Starts with observation as soon as the pt enters the office

• Appearance, gait, behaviors
• Brain and skin have the same embryonic origin: ectoderm
• -Cafe au lait spots, adenoma sebaceum, shagreen patches (genetic d/o on lower back, looks like orange peel)
• Head circumference
• -Premature closure of the suture lines (craniosynostosis) may indicate genetic etiology
• Ocular exam

Question 2

Neonatal exam

Answer 2

Healthy neonates will have periods of quiet and sustained wakefulness
Primative reflexes should be symmetrical when conduction and most resolve in about 4-6 mos
-If they reappear, mass or lesion in neuro system

Question 3

Posture in neonates

Answer 3

Position that a calm infant naturally assumes when supine
28 wks: extended, not a lot of tone
32 wks: trending towards increased tone and flexion
34 wks: upper extended and lower flexed
Full term: flexed upper and lower

Question 4

Movement in neonates

Answer 4

Speeds up with age of infant (premature- slow/full term- faster)

Question 5

Child mental status exam

Answer 5

Alertness
Response to stimuli
Observation of play
Language
-Receptive (understanding speech)
-Expressive (spoken phrases)
Aphasias
-Broca (anterior/expressive): sparse, nonfluent language
-Wernicke (posterior/receptive): inability to understand speech: speech is fluent but non-sensical
-Global: impaired expressive and receptive

Question 6

Cranial nerve child exam: CNs I-VI

Answer 6

I: smell can be assessed like adults starting at age 2+
II: visual acuity is estimated to be 20/200 in newborn and 20/20 by 6 mos
-Swinging flashlight test
–Marcus Gunn pupil
III, IV, and VI: eye movement assessed with something to catch attention
-Infants: dolls eye movement (move head to the right, the eyes move to the left, nl in infants up to 6 mos of age)

Question 7

Child cranial nerve exam: CNs V, VII, VIII

Answer 7

V: mastication muscles observed with swallowing or bottle use
-Corneal reflex tests V and VII
-Facial sensation: light touch, cotton gause, rooting reflex
VII: facial muscle observation or just like adults (puff out cheeks, blink etc)
VIII: neonate- blink with sound, 4 mo-turn to sound, older: whisper a word with other ear covered

Question 8

Child cranial nerve exam: CNs IX-XII

Answer 8

IX and X: gag reflex is brisk at all ages except very immature neonate
-Check gag reflex with tongue depressor
XI: observation of movement
-Drooping of the shoulder and sternocleidomastoid suggests lesions of this nerve
XII: atrophy or fasciculations of the tongue
-Tongue deviates towards the week side in unilateral lesions

Question 9

Motor exam: power

Answer 9

Sponetaneous movements and movements against gravity

Scored 0-5, where 5 is nl

Question 10

Motor exam: tone

Answer 10

Lower motor lesions: decreased tone (hypotonia)
Upper motor lesions: increased tone: spasticity
Extrapyramidal dz: rigidity of a joint

Question 11

Motor exam: what are the other parts of it?

Answer 11

Muscle bulk
Coordination
-Ataxia is defined as the lack of coordination- think cerebellar pathway
Gait
-Typical as a toddler it starts as wide based and unsteady it narrows with age
-By 6 yo should be able to walk on toes, heels, and heel toe walk
Reflexes
-0-4 (2 is nl)

Question 12

What are the most common recurrent pattern of primary HAs in children and adolescents?

Answer 12

Tension HAs

Question 13

Tension HAs: characteristics

Answer 13

Mild
Global
Squeezing
Last for hours or days
No associated nausea, vomiting, photophobia or phonophobia
May be related to environmental stress, anxiety or depression

Question 14

Migraine HAs

Answer 14

Another common type of recurrent HA that often begins in childhood
Frontal, bitemporal, unilateral
Severe pounding or throbbing
Last 1-72 hrs
N/V/photo/phonophobia associated
Toddlers typically appear tired, irritable, pallor color, with vomiting

Question 15

Dx of HAs

Answer 15

In most cases, a good hx and PE provides an accurate dx and nor further testing would be needed
HOWEVER, imaging is warranted when abnl neuro exam is accompanied with the complaints
Focal neurological deficits, alteration of consciousness, or chronic progressive HA pattern
-MRI with and without contrast is study of choice
-Sudden and severe onset, CT should be done quickly
–If CT neg, LP should be done

Question 16

1st line tx for HAs

Answer 16

Symptomatic therapy APAP or NSAID such as ibuprofen or naproxen
Hydration
Anti-emetics for adjunctive therapy

Question 17

2nd line tx for HAs

Answer 17

Triptan class of meds
-CIed in pts with focal deficits or basilar migraines bc of stroke risk

Question 18

Preventative tx for HAs

Answer 18

Must change lifestyle first!!
-Regulate sleep, diet/exercise, counseling, stress management, etc
Antihistamine
TCAs
BBs
CCBs
Anticonvulsants

Question 19

Seizures

Answer 19

Defined as a transient occurrence of s/sx resulting from abnl excessive synchronous neuronal activity of the brain
Approximately 4-10% of children will experience at least one seizure
-Epilepsy is about 1-4%

Question 20

Focal seizures

Answer 20

Simple focal (partial)
-Arise from a specific anatomic focus and location determines sx
-Consciousness is preserved!
Generalized
-Tonic, clonic, biphasic tonic-clonic
–Your typical thought of seizure activity (tonic state then clonic activity leading to a post-ictal phase)

Question 21

Absence seizure

Answer 21

Clinical hallmark is a brief (seconds) loss of environmental awareness accompanied by simple automatisms
Typically start around 4-6 yrs of age
EEG will note a generalized 3-Hz spike and wave activity
Tx: ethosuximide or valproic acid
-Oxcarbamazepine and carbamazepine are relatively contraindicated for children with absence activity as these meds will potentially worsen the activity of the seizure.

Question 22

Febrile seizure

Answer 22

The MCC of seizures among children between 6 mos and 6 yrs of age
Occurs in about 4% of all children
Recur in 30-50% of children, however, prognosis is excellent
Tx is usually related to the infection at hand
Rectal diazepam for prolonged event
Antipyretics will not prevent febrile seizures

Question 23

Juvenile myoclonic epilepsy (of Janz)

Answer 23

MC generalized epilepsy among adolescents and young adults
Onset is typically in early adolescence with myoclonic jerks (typically worse in the morning)
Valproic acid classically used however, many more are effective
Must be on meds for life

Question 24

Infantile spasms

Answer 24

Brief contractions of the neck, trunk, arms followed by a phase of sustained muscle contraction lasting < 2 secs
Most frequently when going to sleep or awakening
Peak age of onset is 3-8 mos
May often be mistaken for colic or acid reflux
ACTH, oral corticosteroid, vigabatrin used for tx

Question 25

Benign neonatal convulsions

Answer 25

Autosomal dominant genetic disorder linked to abnormal K+ channels "Fifth day fits": otherwise healthy baby leading to focal seizures by the end of week 1 Long-term outcome favorable after tx

Question 26

Acquired epileptic aphasia

Answer 26

AKA Landau-Kleffner Syndrome Abrupt loss of previously gained language in young children Language disability is an acquired cortical auditory deficit Should be considered for young pts with clear autistic regression, as potentially treatable entity!

Question 27

Status epilepticus

Answer 27

Emergency Continuous seizure activity for >30 mins when there is no return of consciousness 14% chance of new neuro deficits -Includes scarring, peripheral neuro loss -The longer they go without regaining consciousness, the higher the chance of deficits

Question 28

Status epilepticus tx

Answer 28

First line of tx is establish ABCs Oxygen either administered or through pos pressure IV access and if this is first seizure activity in pt, conduct lab studies Initial management is usually with a Benzo (lorazepam, diazepam, and midazolam all effective) If after 2 doses, move to second line -IV phenytoin, but evaluate for cardiac arrhythmias -3rd line: --Phenobarbital or valproic acid ---4th line: continues medication- general anesthesia

Question 29

Botulism

Answer 29

Results from intestinal infection by Clostridium botulinum This produces a neurotoxin blocking cholinergic transmission This is the most poisonous biological substance known to man

Question 30

Predisposing factor to botulinism

Answer 30

Infants do not have competitive bowel flora | Seen in honey, poorly canned foods, dust or soil with spores

Question 31

S/sx of botulism

Answer 31

Progressive neuromuscular blocks lead to constipation, poor feeding, hypotonia, and weakness, cranial nerve dysfunction (decreasing gag reflex, diminished eye movement, decreased pupillary contraction, and ptosis) Respiratory failure may ensue leading to rapid death

Question 32

Tx of botulism

Answer 32

Therapy with IVIG should be administered as soon as the dx is suspected C. botulinum can be found in stool samples Prognosis is good with tx, respiratory and supportive care

Question 33

Disorders of consciousness

Answer 33

Consciousness is the awareness of self and environment -Mediated by the cerebral cortex Arousal is the system that maintains consciousness -Mediated by the reticular activating system extending from the mid pons through the mid brain and hypothalamus to the thalamus Lethargic means they have difficulty maintaining arousal Obtunded are decreased arousal but can respond to stimuli Stupor means they only respond to pain Coma is not responsive at all Defect in the cerebral hemispheres, brainstem or both

Question 34

What is the MCC of abrupt, episodic loss of consciousness

Answer 34

Syncope 2/3 of children with syncope have irregular, myoclonic movements as they lose consciousness (anoxic seizure) If you can't explain the loss of consciousness, cardiac exam must be completed

Question 35

Metabolic derangements

Answer 35

Hypoglycemia; gives rise to lethargy, confusion, seizures, coma

Question 36

Concussion

Answer 36

Process in which the traumatic forces on the brain result in the rapid onset of short lived neurologic impairment Somatic sx- HA Cognitive sx- poor attention or fogginess Emotional sx- labile or irritable Sleep sx- drowsiness Physical sx- LOC Majority of sx resolve in about 10 days 1 week at least before return to sporting activity Recurrent leads to higher risks of permanent damage

Question 37

Head trauma

Answer 37

Drowsiness, HA, and vomiting are common after head trauma They are not a concern if consciousness is preserved and neuro exam is nl Should sx worsen in 1-2 days imaging is warranted to r/o subdural hematoma or cerebral edema

Question 38

Posttraumatic seizures after head trauma

Answer 38

Impact -Seconds after impact, stimulation of the cortex- prognosis is excellent Early -Within 1 wk of injury, localized area of cerebral contusion or edema- prognosis is favorable Late -More than a wk afterwards, cortical scarring- may be a source of long-term epilepsy

Question 39

Rett syndrome

Answer 39

Classically affects girls Nl development for the first 6-18 mos Followed by a regression, loss of purposeful hand movements, loss of verbal communication skills, gait apraxia, hand motions that typically resemble repetitive washing or clapping Boys will not typically survive to delivery Etiology is linked to the mutation on the X chromosome for MECP2 gene

Question 40

PE of Rett syndrome

Answer 40

Acquired microcephaly is noted | Apnea, peripheral vasomotor disturbances, growth retardation, abnl muscle tone and prolonged QTc interval often occur

Question 41

Spina bifida

Answer 41

Defective closure of the caudal neural tube Range in severity from insignificant to myelomeningocele -Later resulting in flaccid paralysis, loss of sensation in legs and bowel/bladder incontinence A dimple of tuft hair may be present on PE Surgical correction and ventriculoperitoneal shunting for hydrocephalus may be needed Prevention with folate in pregnancy

Question 42

Meningocele

Answer 42

The underlying spinal cord is exposed but functionally and anatomically intact

Question 43

Spina bifida occulta

Answer 43

The skin on back is apparently intact but defects of the underlying bone or spinal cord are present

Question 44

Microcephaly

Answer 44

Less than the 3rd percentile Can be secondary to infections, metabolic, toxic, vascular etiology Sx often present secondary to cause

Question 45

Macrocephaly

Answer 45

Greater than the 97th percentile May be the results of macrocrania- thick skull -Or hydrocephalus- enlargement of the ventricles -Or megaloencephaly- enlargement of the brain Most often, macrocephaly is of no clincial significance and has a familial trait Ensure with each visit that the child is developing normally The brain grows in size until 14 yoa

Question 46

Aseptic meningitis

Answer 46

Viral meningitis with neg CSF | Lyme dz, syphilis, TB, abscess in the brain, NSAID, autoimmune disorders

Question 47

Meningitis

Answer 47

Inflammation of the leptomeninges | Viral, bacterial, fungi (rare)

Question 48

Incidence of meningitis

Answer 48

Very high in children <1 yoa Genetic predisposition may play a role CSF leaks (fistula) increase the risk, esp of S. pneumo Bactrim can be a cause

Question 49

S/sx of meningitis

Answer 49

``` Apnea Stiff or painful neck HA Hearing loss Fever of unknown origin Irritable infant ```

Question 50

Tx of meningitis

Answer 50

``` Empirical cefotaxime (or ceftriaxone) + Vanc should be administered until abx susceptibility testing is available For infants < 2 mos of age: ampicillin is added to cover the possibility of listeria Dexamethasone for 2 days as adjunctive therapy with the first dose of abx diminishes the chance of hearing loss and neuro deficits ```