Neurology Flashcards
Neuro assessment
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
Neonatal exam
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
Posture in neonates
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
Movement in neonates
Speeds up with age of infant (premature- slow/full term- faster)
Child mental status exam
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
Cranial nerve child exam: CNs I-VI
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)
Child cranial nerve exam: CNs V, VII, VIII
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
Child cranial nerve exam: CNs IX-XII
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
Motor exam: power
Sponetaneous movements and movements against gravity
Scored 0-5, where 5 is nl
Motor exam: tone
Lower motor lesions: decreased tone (hypotonia)
Upper motor lesions: increased tone: spasticity
Extrapyramidal dz: rigidity of a joint
Motor exam: what are the other parts of it?
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)
What are the most common recurrent pattern of primary HAs in children and adolescents?
Tension HAs
Tension HAs: characteristics
Mild
Global
Squeezing
Last for hours or days
No associated nausea, vomiting, photophobia or phonophobia
May be related to environmental stress, anxiety or depression
Migraine HAs
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
Dx of HAs
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
1st line tx for HAs
Symptomatic therapy APAP or NSAID such as ibuprofen or naproxen
Hydration
Anti-emetics for adjunctive therapy
2nd line tx for HAs
Triptan class of meds -CIed in pts with focal deficits or basilar migraines bc of stroke risk
Preventative tx for HAs
Must change lifestyle first!! -Regulate sleep, diet/exercise, counseling, stress management, etc Antihistamine TCAs BBs CCBs Anticonvulsants
Seizures
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%
Focal seizures
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)
Absence seizure
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.
Febrile seizure
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
Juvenile myoclonic epilepsy (of Janz)
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
Infantile spasms
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
Benign neonatal convulsions
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
Acquired epileptic aphasia
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!
Status epilepticus
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
Status epilepticus tx
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
Botulism
Results from intestinal infection by Clostridium botulinum
This produces a neurotoxin blocking cholinergic transmission
This is the most poisonous biological substance known to man
Predisposing factor to botulinism
Infants do not have competitive bowel flora
Seen in honey, poorly canned foods, dust or soil with spores
S/sx of botulism
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
Tx of botulism
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
Disorders of consciousness
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
What is the MCC of abrupt, episodic loss of consciousness
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
Metabolic derangements
Hypoglycemia; gives rise to lethargy, confusion, seizures, coma
Concussion
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
Head trauma
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
Posttraumatic seizures after head trauma
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
Rett syndrome
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
PE of Rett syndrome
Acquired microcephaly is noted
Apnea, peripheral vasomotor disturbances, growth retardation, abnl muscle tone and prolonged QTc interval often occur
Spina bifida
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
Meningocele
The underlying spinal cord is exposed but functionally and anatomically intact
Spina bifida occulta
The skin on back is apparently intact but defects of the underlying bone or spinal cord are present
Microcephaly
Less than the 3rd percentile
Can be secondary to infections, metabolic, toxic, vascular etiology
Sx often present secondary to cause
Macrocephaly
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
Aseptic meningitis
Viral meningitis with neg CSF
Lyme dz, syphilis, TB, abscess in the brain, NSAID, autoimmune disorders
Meningitis
Inflammation of the leptomeninges
Viral, bacterial, fungi (rare)
Incidence of meningitis
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
S/sx of meningitis
Apnea Stiff or painful neck HA Hearing loss Fever of unknown origin Irritable infant
Tx of meningitis
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
