Neurology Flashcards
1
Q
arbovirus pattern
A
warm climates, carried by insects (west nile, St. Louis)
2
Q
enterovirus pattern
A
generalized neurological findings, transmitted human to human
3
Q
herpes encephalitis diagnostic test
A
DNA PCR
4
Q
frontal headache with bandlike pressure
A
stress/tension headache
5
Q
episodic severe headaches that cause a child to stop their activities and lay down
A
migraine
6
Q
migraine headache treatment
A
ibuprofen, Tylenol, fluids and rest
ergotamine and sumatriptan if needed
7
Q
migraine headache prevention
A
cyproheptadine or topiramate
8
Q
contraindicated when signs of increased ICP
A
LP
9
Q
diagnostic test of choice when concerned for increased ICP
A
CT with contrast urgently, MRI once stable
10
Q
increased ICP of unknown etiology aka
A
psuedotumor cerebri
11
Q
untreated pseudotumor cerebri leads to
A
papilledema –> optic disk atrophy –> blindness
12
Q
double vision in pseudo tumor cerebri is 2/2 to
A
6th nerve palsy
13
Q
headache in pseudotumor cerebri gets worse with
A
laying flat and valsalva
14
Q
common cause of pseudotumor cerebri
A
megadose vitamin intake, especially vitamin A
15
Q
eye findings in infants with increased ICP
A
setting sun/downward deviation
16
Q
medications that can cause pseudotumor cerebri
A
vitamin A, steroids, thyroxine, lithium, some antibiotics
17
Q
psudotumor cerebri treatment
A
acetazolamide (carbonic anhydrase inhibitor), severe cases require steroids and surgery/shunt
18
Q
Cushing’s triad
A
HTN, bradycardia, abnormal respirations (late findings of increased ICP/impending herniation)
19
Q
major risk factor for hydrocephalus
A
neural tube defect (chair malformation or myelomeningocele)
20
Q
most shunt infections occur when
A
within 6 months of placement
21
Q
presentation of shunt malfunction
A
symptoms of increased ICP but without fever
22
Q
common location of brain tumors in kids
A
posterior fossa
23
Q
most common type of solid tumors in kids
A
brain tumors
24
Q
contrast enhancing 4th ventricle mass
A
medulloblastoma
25
most common malignant brain tumor of childhood
medulloblastoma
26
medulloblastoma presentation
headache, ataxia, obstructive hydrocephalus
27
presentation of posterior fossa tumors
headache, ataxia, head tilt, torticollis, vomiting
28
supratentorial tumors
cranioopharyhngioma, optic nerve glioma
29
craniopharyngioma presentation
slow progression with triad of endocrinopathies (DI, short stature), visual disturbances and headaches
30
cause of chronic visual field deficits in craniopharyngioma
pressure on the optic tracts
31
cause of hydrocephalus in craniopharyngioma
obstruction of the 3rd ventricle
32
craniopharyngioma treatment
surgery +/- radiation
33
optic nerve gliomas have a 25% association with
neurofibromatosis
34
patients with what disease have an increased risk for brain abscess
pulmonary, sinus and cyanotic heart disease
35
CT description of brain abscess
ring enhancing lesion
36
most common cause of sinusitis
S. aureus
37
common treatment for brain abscess
nafcillin/vancomycin, metronidazole and ceftriaxone
38
cause of cranial bruit in a neonate with hydrocephalus and CHF
vein of Galen malformation
39
presentation of younger patients with Wilson's disease
liver disease
40
presentation of older patients with Wilson's disease
neuro/psych symptoms
41
diagnosis with acute hepatic failure, dystonia and mental status changes
wilson's disease
42
sudden drops precipitated by sudden emotion such as laughter
cataplexy
43
diagnosis of narcolepsy
overnight polysomnography and sleep latency test
44
acute onset ataxia is most likely 2/2
post-vital
45
ataxia telangiectasia findings
CNS/sometimes intellectual disability, skin and eye findings from capillary dilations, decreased IgA, IgG and T-cell function causing frequent upper and lower respiratory tract infections
46
advanced ocular telangiectasias cause
inability to voluntarily make rapid eye movements (pupillary reflexes and acuity usually are normal)
47
high risk of what with ataxia telangiectasia
malignancy - especially Hodgkin lymphoma and leukemia
48
ataxia telangiectasia inheritance
autosomal recessive
49
Friedrich ataxia inheritance
autosomal recessive
50
friedreich ataxia presentation
slow and clumsy gait in late childhood or early adolescence
51
cause of ataxia in friedreich ataxia
cerebellar component and loss of proprioception
52
clinical features that distinguish Friedreich ataxia
elevated plantar arch, no lower extremity DTRs, cardiomyopathy leading to CHF
53
relatively rapid random repetitive purposeless movements
chorea
54
part of the brain most affected in movement disorders
basal ganglia
55
Sydenham chorea association
major Jones criteria for rheumatic fever
56
Huntington's disease triad
chorea, hypotonia and emotional lability
57
juvenile Huntington's disease presents most often with
rigidity rather than hypotonia
58
chorea treatment
dopamine blocking agents ex: haloperidol (also can use fluphenazine, risperidone or tetrabenazine)
59
treatment of dystonic reaction
diphenydramine
60
neck hyperextension and decreased extra ocular movements
dystonic reaction
61
meds that can cause dystonic reactions
neuroleptics ex: metoclopramide or promethazine
62
choreiform movements vs tics
choreiform movements get worse during purposeful movement and cannot be voluntarily suppressed whereas tics improve during purposeful movements and can be suppressed
63
Tourette syndrome diagnosis length
1 year of symptoms
64
location of lesion with weak eye movements, ipsilateral facial weakness and contralateral body weakness
brainstem lesion
65
location of lesion with loss of motor and sensation, lord of bladder/bowel, increased reflexes
spinal cord lesion
66
test if concerned for spinal cord lesion
MRI spine
67
acute transverse myelitis aka
post infectious myelitis
68
cause of acute transverse myelitis
lymphocytic infiltration and demyelination of the nerves/spinal cord 2/2 inflammation
69
CSF findings in acute transverse myelitis
increased polys and negative gram stain
70
MRI findings in acute transverse myelitis
swelling of the spinal cord
71
main concern in acute transverse myelitis
respiratory compromise/arrest
72
test that must be preformed first with acute transverse myelitis
MRI before LP
73
abrupt onset of weakness, hypotonia and decreased reflexes followed by increased tone and hyperreflexia
acute transverse myelitis
74
fever and sudden onset of paralysis
acute transverse myelitis
75
treatment for extrinsic spinal cord mass lesion
high dose IV dexamethasone
76
werdnig-hoffman disease aka
SMA type 1
77
mechanism of werdnig-hoffman disease
degeneration of anterior horn cells - only motor function is affected
78
diagnosis of wedding-hoffmann disease
muscle biopsy
79
hypotonia, poor suck and tongue fasciculations
werdnig-hoffman disease (SMA type 1)
80
finding that distinguishes SMA from botulism
tongue fasciculations
81
when in development do neural tube defects occur
between weeks 3 and 4
82
things that increase the risk for neural tube defects
maternal folate deficiency and maternal anticonvulsant use
83
who needs consulted if you suspect neural tube defects in a newborn
ortho and urology
84
early subtle findings of occult spinal dysraphism
lipoma, patch of hair, hemangioma, discoloration or sacral dimple
85
late findings of occult spinal dysraphism
leg length discrepancy, elevated arches, gait abnormalities
86
management for suspected spinal trauma
embolization and methylprednisolone 30 mg/kg over 1 hour
87
acquired idiopathic unilateral facial paralysis 2/2 post viral inflammation of the 7th CN
bell's palsy
88
is forehead involved in bell's palysy
includes forehead weakness (decreased wrinkling)
89
unilateral facial weakness that spares the forehead
evaluate for stroke
90
treatment for bell's palsy
prednisone x5 days (works best if started within 3 days of symptom onset) or acyclovir in severe cases
91
facial paralysis associated with painful vesicles in the ear canal
Ramsay hunt syndrome (reactivation of varicella zoster)
92
progressive proximal motor weakness and areflexia that progresses from lower to upward symmetrically
Guillain barre syndrome
93
what is always present in guillain barre syndrome
areflexia
94
physical exam findings in Guillain barre
CN findings na dysautonomia (tachycardia, orthostatic hypotension, dizziness)
95
CSF findings of Guillain barre sydrome
increased protein, normal cell count
96
Guillain barre treatment
supportive plus plasmapheresis and IVIG
97
autoimmune disorder with antibodies against the acetylcholine receptor in the neuromuscular junction
myasthenia gravis
98
what presents similarly to Guillain barre
tick paralysis during the summer months
99
characteristics of weakness with myasthenia gravis
weakness gets worse with activity and improves with rest
100
common first presentation of myasthenia gravis
ptosis
101
myasthenia gravis is associated with
thymoma
102
test to confirm myasthenia gravis
tensilon test (uses edrophonium, a short acting acetylcholinesterase inhibitor)
103
chronic treatment for myasthenia gravis
pyridostigmine (inhibits acetylcholinesterase to increase amount of acetylcholine available at the neuromuscular junction)
104
treatment that sometimes works to cure myasthenia gravis
thymectomy
105
MOA of botulism toxin
blocks the release of acetylcholine from the neuromuscular junction
106
type of paralysis with botulism
descending paralysis
107
cause of Duchenne muscular dystrophy
absence of dystrophin
108
how to diagnose Duchenne muscular dystrophy
muscle biopsy
109
what is more severe in Becker compared to Duchenne's
cardiac involvement
110
genetic testing for Duchenne muscular dystrophy
Xp21 gene
111
finding in asymptomatic female carrier of Duchenne muscular dystrophy
elevated CPK
112
myotonic muscular dystrophy affects what kind of muscle
smooth and striated muscles
113
myotonic muscular dystrophy presentation
muscles that have slow relaxation after contraction, usually presents around 4 to 5 years old
114
type of muscle wasting in myotonic dystrophy
distal
115
myotonic muscular dystrophy inheritance pattern
autosomal dominant
116
sudden electrical discharge of nerve cells from the gray matter that progresses to the white matter
seizure
117
status epilepticus
seizure >30 minutes or repeat seizure without returning to normal in between
118
age range for febrile seizures
6 months to 5 years
119
simple febrile seizure
generalized seizure that lasts less than 15 minutes and only once within a 24 hour time period
120
increased risk for developing epilepsy with febrile seizures if
first febrile seizure is complex
121
complex febrile seizure
lasts longer than 15 minutes, recurs within 24 hours or focal
122
brief staring spells/non responsive during this time without post octal period
absence seizure
123
3 per second spike and wave on EEG
absence seizure
124
diagnostic maneuver for absence seizure
hyperventilation
125
absence seizure treatment
ethosuximide, lamotrigine or valproic acid
126
prognosis with absence seizure
younger the child the better response to treatment
127
monitoring that needs done with valproic acid
watch for thrombocytopenia, elevated LFTs and elevated pancreatic enzymes
128
tonic phase
flexion of trunk, extension of back, arms and legs, no loss of bladder control, lasts ~30 seconds
129
clonic phase
clonic convulsive movements with tremors, short periods of atonia where there is loss of bladder and apnea
130
drug of choice for generalized tonic clonic seizures
valproic acid
131
potential side effect of lamotrigine
Steven Johnson's
132
EEG and juvenile myoclonic epilepsy
usually normal
133
juvenile myoclonic epilepsy presentation
myoclonic jerks upon waking, most have GTCs some have absence seizures`
134
juvenile myoclonic epilepsy treatment
valproic acid or levetiracetam
135
west syndrome aka
infantile spasms
136
west syndrome triad
infantile spasms, hypsarrhythmia on EEG, developmental delay
137
typical time of presentation with infantile spasms
3 to 9 months
138
sudden flexion or extension of the body
infantile spasm
139
association between infantile spasms and
tuberous sclerosis
140
first line treatment of infantile spasms
ACTH (others are steroids, benzodiazepines, focal resection)
141
prognosis with infantile spasms
depends on developmental status prior to spasms starting
142
simple in seizures means
consciousness maintained
143
partial in seizures means
focal
144
simple partial seizure treatment
carbamazepine (many go away by adolescence or don't need treatment)
145
benign rolandic epilepsy aka
benign childhood epilepsy with centrotemporal spikes
146
most common partial epilepsy of childhood
benign rolandic epilepsy
147
self limited seizures involving the face
benign rolandic epilepsy
148
benign rolandic epilepsy is associated with
migraines
149
benign rolandic epilepsy most often occurs
at night
150
treatment for benign rolandic epilepsy
only if 3 or more episodes
151
prognosis of benign rolandic epilepsy
usually resolve by age 16
152
benign Rolandic epilepsy during the ay
fully aware but unable to speak and have unilateral sensory involvement (usually one side of the tongue)
153
benign rolandic epilepsy EEG findings
biphasic focal centrotemporal spikes and slow waves
154
treatment of seizure if Ativan doesn't work
fosphenytoin
155
can teens with seizures drive
if on antieplieptic and seizure free x 12 months
156
when can you try to stop antiepileptics
if seizure free x2 years
157
most common cause of CP in preemies
perinatal infection
158
CP with bilateral leg spasticity, delayed walking and tip toe walking
spastic diplegia
159
CP with spasticity of one half of the body (arm, trunk and leg of that side)
spastic hemiplegia
160
CP with increased tone in all 4 extremities (L>U)
spastic quadriplegia
161
CP with increased tone in all 4 extremities, dystonia and strange movements
dyskinetic
162
next step with CVA confirmed on CT
cerebral angiography
163
history consistent with stroke and has elevated lactate and sensorineural deafness
mitochondrial disease - get a molecular analysis of DNA
164
most common cause of hemorrhagic stroke in children
vascular malformations
165
diagnosis of infant with sever seizures and CT showing bilateral clefts within the cerebral hemispheres
schizencephaly
166
friederich ataxia chromsome
9
167
diagnosis of newborn with herniation of the brain and its coverings through a skull defect in the occipital region
encephalocele
168
diagnosis in newborn with herniation of meninges through defect in posterior vertebral arches, spinal cord is normal and area is well covered with skin
meningocele
169
most common abnormalities that develop in kids with myelomeningocele
hydrocephalus and chiari II malformation
170
diagnosis of newborn with severe seizures and CT shows brain without cerebral convolutions and poorly formed Sylvian fissure
lissencephaly
171
diagnosis with prenatal brain US showing widely separated frontal horns w/ 3rd ventricle high between the lateral ventricles, mom has used cocaine
agenesis of the corpus callosum
172
most likely prothrombotic factor to cause an ischemic stroke in a child
acquired antiphospholipid antibody
173
most common inheritable cause of a stroke
activated protein C resistance (factor 5 Leiden)
174
extensive collateral vessels from prior occlusion around the circle of willis on cerebral angiography of child with sickle cell
moyamoya
175
what arterial distribution do most neonatal cerebral infarctions occur
left middle cerebral artery
176
most common location for skull fractures in kids
parietal
177
EEG pattern with generalized bilateral synchronous sharp wave and slow wave complexes occurring in a repetitive fashion in long runs at ~2 Hz
Lennox gastaut
178
diagnosis with history of focal unremitting seizures on L side of the body with progressive hemiparesis on that side and atrophy of the R hemisphere on MRI
Rasmussen syndrome
179
type of migraine with vertigo, syncope, dysarthria, visual alterations and LOC
basilar artery migraine
180
diagnosis with worsening muscle weakness exacerbated by repetitive muscle use an ocular muscle weakness, ptosis
juvenile myasthenia gravis
181
diagnosis with optic neuritis, oculomotor disturbances, incoordination of upper extremities, sensory deficits, CSF with IgG and oligoclonal bands, MRI with areas of active demyelination and evidence of prior demyelination
multiple sclerosis
182
SMA type 1 is carried on what gene/chromosome
SMN1 gene on chromosome 5q13
183
how to confirm diagnosis of Duchenne muscular dystrophy
identify mutation on the DMD gene (if negative and high suspicion then muscle biopsy)
184
diagnosis in newborn of mom with a neurologic disease that at birth has hypotonia, weak cry, difficulty feeding, facial weakness, ptosis and respiratory distress
neonatal myasthenia gravis from transplacental transmission of maternal acetylcholine receptor antibodies (AChR-Ab) - resolves in 2 to 6 weeks
185
treatment for juvenile myasthenia gravis
oral anticholinesterase meds (pyridostigmine)
immunosuppression
thymectomy
plasmapheresis or IVIG
