Nelson Essentials Neuro Flashcards
1
Q
Skin features of tuberous sclerosis
A
- adenoma sebaceum - fibrovascular lesions that look like acne on nose and male regions
- nail fibromas
- ash-leaf spots - hypo pigmented macules
- Shagreen patches
2
Q
What is Broca aphasia
A
anterior, expressive aphasia
sparse, confluent language
3
Q
What is Wernicke aphasia
A
receptive, posterior aphasia
inability to understand language, with speech that is fluent but nonsensical
4
Q
What is vision in newborns? in older infants?
A
should be able to follow face in a dark room
20/200 in newborns, 20/20 in 6 month olds
5
Q
What is an afferent pupillary defect
A
swinging flashlight test - light on the abnormal eye, both pupils dilate inappropriately; on the normal eye, they both constrict
6
Q
What is Horner syndrome
A
meiosis
anhydrosis
ptosis
7
Q
What is the doll’s eye maneuver?
A
rotate the infant’s head - > if brainstem is okay, then moving the head of newborn or comatose patient, eyes will move to the left and vice versa
in awake, older patients, voluntary eye movements mask the reflex
8
Q
What does the eye look like in oculomotor 3rd CN palsy?
A
eye will be down and out with ptosis and dilated pupil
9
Q
CN IV palsy?
A
weakness of downward eye movement
vertical diplopia
10
Q
CN VI palsy
A
can’t move the eye outward - therefore the eye will be inward
has a long intracrnial route - a frequent sign of increased ICP (but nonspecific)
11
Q
Which cranial nerves are tested by corneal reflex?
A
V opthalmic division and VII at agy age
facial sensation
12
Q
weakness of a patient’s face, only affects the lower face and mouth? where is the lesion?
A
consider upper motor neuron lesion - tumor/stroke abscess
-
13
Q
weakness of an entire side of the face?
A
CN 7 palsy - Bell palsy -
14
Q
True or false - very premature neonates should have a gag reflex
A
very immature neonates may not have but all other ages should have a brisk gag
15
Q
atrophy and fasciculation of the tongue, what type of lesion to think of?
A
anterior horn cell problem - i.e. in SMA
most reliable when baby asleep
tongue will deviate toward the weak side in unilateral lesions
16
Q
Conditions with decreased muscle bulk
A
lower motor neurone conditions - neuropathies, SMA
muscle bulk is diminished or atrophic
17
Q
Conditions with increased muscle bulk
A
- myotonia congenita
pseudo hypertrophy of calves - muscular dystrophy
18
Q
Ways to access cerebellar dysfunction?
A
- ataxia
- intension tremor
- dysmetria
19
Q
When should the Babinski be down going?
A
12-18 months
20
Q
DTRs in lower motor neurone vs upper?
A
decreased in LMN, increased in UMN
21
Q
What does muscle fibrillation/fasciculation indicate?
A
indicates denervation of muscles/nerves
22
Q
Abnormal muscle response to repetitive nerve stimulation, Differential?
A
- abnormal response to repetitive stimulation of nerve: Neuromuscular conditions: M. gravis and botulism
- lower amplitude and duration of muscle action potentials - in primary muscle diseases
- slowed nerve conduction velocities - demyelinating nerve conditions - i.e. GBS
- lower amplitude of signal - decreased in axonal neuropathies
23
Q
new onset of complex partial seizures, what type of imaging to do?
A
MRI - may not see areas of focal cortical dysplasia or other subtle lesions , may not be apparent on CT
24
Q
Most common type of recurrent primary headache in children and teens
A
tension types headaches - most common type of recurrent primary headaches in children and teens
pain is global and squeezing
can last hours to days
no associated nausea, vomiting, phono phobia or photophobia
25
Migraine headaches
stereotyped attacks of frontal, bitemporal or unilateral moderate to severe, pounding or throbbing pain aggravated by activity
lasts 1-72 hours
associated symptoms: nausea, vomiting, pallor, photophobia, phono phobia, desire to seek a quiet dark room for rest
can be associated with auras
complex, atypical symptoms with migraine - i.e. hemiparesis, monocular blindness, ophthalmoplegia or confusion need investigations
26
Most common causes of secondary headaches -
1. head trauma
2. intercurrent viral illness
3. sinusitis
4. medication overuse headaches
5. increased ICP - if worse h/a lying down or when awake , worse when coughing/valsalva, or pending over
27
which pattern or headache most concerning
chronic progressive
need to image
MRI is the best - since better to detect posterior fossa lesions
28
Treatment of migraines:
symptomatic or abortive tretament - early analgesia, rest and sleep
acetaminophen/NSAID
hydration and anti-emetics
if these don't work, then consider triptans - serotonin receptor agonists
29
contraindications to triptans
focal neuro deficits with migraine
| signs consistent with basilar migraine - syncope - because of risk of stroke
30
Preventative treatments for migraines
can consider when >1 disabling headache per week
TCAs - ie amitriptylline, nortriptyline
anticonvulsants - topiramate, valproic acid
antihistamines - cyproheptadine
beta-blockers - propanolol
calcium channel blockers - flunarizine, verapamil
before these; lifestyle modification : sleep, routines, exercise, identify precipitating factors (i.e. caffeine, foods, stress, meals missing, dehydration
psych/stress management/biofeedback are other things to consider
31
EEG pattern in absence seizure
3hz spike and wave
can have multiple in one day
should be back to self ASAP
32
what age does absence seizure usually start
4-6 year old
| can provoke with lights and/or hyperventilation
33
BECTs
usually age 5-10 year old
seizures usually during sleep or on awakening in most patients
focal motor seizures involving face and arm - abnormal movement/sensation, drooling, guttural sound
may have speech arrest/swallowing problem
sometimes get secondary generalization
34
BECTS - EEG findings and , do you need imaging?
imaging (interictal _ bilateral centrotemporal sharp waves but otherwise nomal
if otherwise normal neuro exam - then don't need imaging
intellectual outcome normal, resolves after puberty
35
BECT comorbidities
ADHD/learning difficulties
36
Absence epilepsy - if doesn't remit, what type of epilepsy might they develop
juvenile myoclonic epilepsy
37
patient with absence epilepsy and tonic - clonic seizures, 1st choice treatment?
valproic acid, can help with both
38
classic treatment for juvenile myoclonic epilepsy
valproic acid (but other meds also have been showed
most common generalized epilepsy among teens and young adults
myoclonic jerks in the AM
generalized tonic-clonic seizures
absence seizures
39
when do infantile spasms happen and how long do they last usually?
usually <2 seconds
most when awakening or going to sleep
often have clusters
West syndrome: infantile spasms, developmental regression, abnormal EEG pattern (hypsarrhthmia)
age of onset: 3-8 months is the peak **may be
40
Differential for infantile spasms
1. tuberous sclerosis
2. malformations of cortical development
3. genetic syndromes - i.e. trisomy 21
4. acquired brain injury - i.e. stroke, HIE
5. metabolic disorders - ie PKU
41
better prognosis for symptomatic infantile spasms or from cryptogenic?
cryptogenic somewhat better than symptomatic (i.e. those who have an underlying aetiology)
42
Treatment of infantile spasms
ACTH treatment, steroids, vigabatrin
| for patients with tuberous sclerosis - whose vigabatran
43
What is Lennox-Gastaut syndrome
severe spilepsy syndrome
most kids present before 5 years old
multiple seizure types - including atonic, focal, atypical absence, generalized tonic, clonic or tonic-clonic varieties
big Nelson says:
between age 2-10 years, developmental delay, multiple seizure types
tonic seiuzures occur while awake or while asleep
EEG findings: 1-2 Hz spike and slow waves
most are left with significant MR
44
What is Landau-Kleffner syndrome
abrupt loss of previously acquired language
EEG highly epileptiform
peak area of abnormality in dominant perisylvian region
should consider in kids who have clear autistic regression
Big nelson:
rare condition of unknown cause
hearing is nona
have behavioural problems
lots of types of seizures
EEG findings more apparent in sleep- if you suspect this, need to do EEG during sleep
Treatment: valproic acid
onset at early age - poor prognosis
45
What are benign neonatal convulsions
autosomal dominant genetic disorder - abnormal neuronal potassium channels
otherwise well newborns - focal seizures towards 5 days of age - "fifth-day fits"
usually have good response to treatment , favourable long-term outcome
46
focal seizures - best drugs
oxcarbaepine, carbamazepine (big nelson)
| little nelson also says phenytoin, lacosamide
47
best med for absence seizures
ethosuximide
48
juvenile myoclonic epilepsy - best med
valproate and lamotrigine
49
best drug for lennox-gastaut
valproate topiramate, lamotrigine
50
generalied epilepsy
valproate has been shown to be effective
51
main side effect of vigabatrin
retinal toxicity
52
baby nelson says for generalized seizures
clobazam, felbamate
53
for both focal and generalized
lamotrigine, levetiractam, phenobarbital, topiramate, valproic acid, zonisamid, ketogenic diet
54
side effects of valproate therapy
main one is liver toxicity in kids < 2year old, on polytherapy or with metabolic disorders , need to rule out metabolic disease
55
baby with tongue fasciculations, absent DTRs, rapid/shallow/abdominal breathing -likely diagnosis
SMA type 1
(the other types of SMA present later and more gradually)
inheritance if autosomal recess
56
how to diagnose SMA 1
diagnosis by genetic testing
CK may be normal or mildly elevated
EMG - fasciculations, fibrillations, other signs of denervation
muscle biopsy show grouped atrophy
57
Treatment of SMA
minimize contractures, prevent scoliosis, maximizing nutrient, avoiding infections
58
Most classic presentations of peripheral neuropathy in childhood
1. GBS
2. chronic inflammatory demyelinating polyneuropathy
3. hereditary motor sensory neuropathy
4. tick paralysis
59
Most common cause of acute flaccid paralysis in children
gbs
Mycoplasma and Campylobacter cause it
post infectious autoimmune peripheral neuropathy
symptoms: areflexia, flaccidity and symmetrical ascending weakness
progression in hours, or over weeks
weakness can ascend to involve the arms, trunk and bulbar muscles
DTRs ABSENT (even if have some strength)
can get autonomic dysfunction
60
what is miller fischer variant of Guillain-Barre?
ataxia
partial opthalmoplegia
areflexia
61
What is the classic CSF finding in Guillain barre?
can be normal early in illness
elevated protein levels without significant pleocytosis
MRI - may show enhancement of spinal nerve roots
62
Treatment of Guillain Barre
IVIG ; alternatives are plasma exchange dna immunosuppressive therapies
63
When does Guillain Barre resolve
80% back to normal in 1-12 months
| can have relapse
64
Charcot Marie Tooth
start with frequent tripping/ankle weakness
will have pea caves deformity
eventually get weakness and atrophy of lower legs and hands, get mild to moderate sensory loss in hands and feet
no specific treatment
65
Treatment of juvenile M. gravis?
pyridostigmine - inhibits acetylcholinesterase
| if severe, may need immunosuppression
66
Treatement of neonate born to mother with myasthenia gravis - who presents with ptosis, opthalmoplegia, weak facial movements, poor feeding, hypotonia, respiratory difficulty, variable extremity weakness
cholinesterase inhibitors, supportive care
67
Congenital M. Gravis vs. juvenile
juvenile - variable ptosis, diplopia, ophthalmoplgia, facial weakness
worse when tired, with repetitive activity
some kids, never advances, others will have effect on resp and swallowing
vs. congenital - is a gene mutation in the neuromuscular junction ; might have resp problems, can have lifelong problems - some will respond to pyridostigment
68
Diagnosis fo M. gravis
electrodecrement with 3hz repetitive stimulation
most will have antibodies to the acetylcholine receptor
"Tensilon" test - edrophonium chloride - cholinesterase inhibitor - can lead to transient improvement i strength (especially of ptosis)
69
infantile botulism
presentation - constipation and poor feeding
then get hypotonia and weakness, CN dysfunction
can get resp failure
70
Treatment of infantile botulism:
botulism IVIG
71
Symptoms of muscular dystrophy
1. awkward gait, +ve Gower sign
2. cardiomyopathy
3. scoliosis
4. resp decline
5. some boys have cognitive and behavioural dysfunction
72
Labs in Muscular dystrophy?
elevated CK
genetic testing of dystrophin gene
can do prenatal diagnosis
1/3 of cases are new mutations
73
inheritance of myotonic dystrophy
autosomal dominant genetic disease - progressive expansion of triplet repeat
genetic anticipation - each generation is worse than the previous
74
Clinical features of myotonic dystrophy
1. weakness - progressive facial and DISTAL extremity weakness
2. myotonia: muscles have a hard time relaxing after contraction
3. characteristic facial appearance
4. cardiac muscle - arrythmias (can have second wave of arrythmias in teens, can kill ya)
5. endocrinopathies
6. immunologic deficiencies
7 cataracts
8. intellectual impairment
*severe congenital myotonic dystrophy - can also have some atony of uterus, present with HIE makes diagnosis even harder
75
prenatal features which can suggest intrauterine neuromuscular disease
contractures
clubfoot
poor fetal movements
76
Malignant hyperthermia
1. increase temperature, muscle rigidity, metabolic and respiratory acidosis, hypotension, arrythmias, convulsions
2. can have increase in CK then myoglobinuria, result in ATN, acute renal failure
Diagnosis: genetic or in vitro muscle contraction
77
Treatment of malignant hyperthermia
IV dantrolene
bicarb
cooling
78
most common cause of hypotonia
HIE
79
most common genetic causes of neonatal hypotonia
PWS, trisomy 21
failure to thrive, small hands and feet
in boys, small penis, small testicles and cryptorchidism
80
What is benign congenital hypotonia
age 6-12 months, delay gross motor
family history
seem floppy from birth
usually catch up by age 3
81
pediatric stroke management
thrombolytic not proven
| anticoagulation if progressive
82
most common causes of acute ataxia in children
drug intoxication
post infectious acute cerebellar ataxia
other causes: tumours, MS, strokes, bleeds , BPPV, head trauma, seizures, postictal states, migraine, paraneoplastic opsoclous-myoclonus syndrome with neuroblastoma, inborn errors of metabolism
83
Ingestions for ataxia with lethargy and without?
ataxia with lethargy : sedative-hypnotic agent
| without lethargy: ethanol or anticonvulsant intoxication
84
What is the difference between truncal and appendicular ataxia?
truncal ataxia: disturbance of midline cerebellar vermis - ie medulloblastoma, acute postinfectius cerebellar ataxia, ethanol ingestion
Appendicular ataxia: disturbance of the ipsilateral cerebellar hemisphere
85
usual symptoms of ataxia
broad based unsteady gait (truncal ataxia)
intention tremor or dysmetria
usually from cerebellar pathways, but CAN have from peripheral nerve lesions which cause loss of proprioceptive inputs to cerebellum (i.e. GBS)
86
mitochondrial diseases which can present with ataxia and somnolence?
```
Harnup
maple syrup urine
mitochondrial
abetalipoproteinemmia
vitamin E deficiency
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